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Introduction: Echinococcus multilocularis is a small but deadly tapeworm species found worldwide that can cause a disease called Alveolar Echinococcosis (AE) in humans and animals (Gottstein et al., 2015; Kolapo et al., 2023; Meyer et al., 2020). AE is a rare zoonotic disease that typically affects the liver of a susceptible host and can be fatal if left untreated (Meyer et al., 2020; WOAH, 2022). E. multilocularis normally occurs in predator-prey cycles of wild definitive hosts (e.g. foxes and coyotes) and intermediate hosts (e.g. rodents; WOAH, 2022). AE occurs in intermediate hosts when eggs are ingested from the environment and are transported to the liver where the metacestode (larval) stage of the parasite proliferates (Lundström-Stadelmann et al., 2025). However, AE has been found in aberrant intermediate hosts (e.g. humans, dogs) increasingly in recent years in both North America and Europe (Jenkins et al., 2025). Currently, most human cases of AE (greater than 90%) are reported in Asia (Torgerson et al., 2010), with most others reported in Europe, where there has been an increase in the parasite’s prevalence and endemic range expansion since 2001 (Jenkins et al., 2025). Prior to 2009, there were only two North American cases of AE in humans (Manitoba 1928, Minnesota 1977; Gamble et al., 1979; James & Boyd, 1937), and none reported in dogs (Jenkins et al., 2025; Kolapo et al., 2023). Similar to trends observed in Europe, animal AE cases (since 2009) and human AE cases (since 2013) have been increasing at a concerning rate, whilst expanding the range of historically endemic areas (Deplazes et al., 2017; Jenkins et al., 2012; Massolo et al., 2014). This increase in prevalence of AE cases could be attributed to the introduction and virulence of the European strain of E. multilocularis (Jenkins et al., 2025; Luong et al., 2020). The global rise of AE cases poses a threat to human and animal health and requires a One Health approach to appropriately coordinate research efforts to respond to this emerging disease. Identification of risk factors of AE could aid in reducing exposure to and transmission of the parasite, as well as early detection of infection. Given the sudden emergence of AE affecting humans and other atypical animal hosts in North America, there is an urgent need to understand the epidemiology of AE. In other endemic parts of the world, such as Asia and Europe, the epidemiology of AE is better understood and could help inform the unprecedented situation in North America. Therefore, to address the knowledge gap in exposure and host risk factors associated with AE in humans and animals, we will identify and synthesize current knowledge from peer-reviewed scientific publications and grey literature globally. Objective: The objective of this review is to identify, assess, and synthesize current knowledge on host and exposure risk factors associated with AE in humans and animals in the peer-reviewed scientific literature, theses and dissertations, and grey literature globally using a One Health framework. Methods: Relevant article databases will be searched for literature on either E. multilocularis or AE in human or animal species. ProQuest Dissertations & Theses global will also be searched to identify relevant dissertations. Additional grey literature sources will be identified through expert consultation to identify data collection tools currently in use for human AE cases. There will be 2 stages that comprise the article screening process. The first stage of screening will be title and abstract, while the second stage is full text. The initial search will include concepts of E. multilocularis and AE in both humans and animals. Inclusion of articles in this review will be peer-reviewed scientific literature (case studies/reports, review articles, and journal articles) and grey literature (data collection tools identified through consultation with experts, conference proceedings, and theses/dissertations) discussing AE caused by infection of E. multilocularis in humans and animals. Any source that clearly is unrelated to risk factors for AE or E. multilocularis will be excluded at the title/abstract stage of screening. Literature that focuses solely on the intestinal form of E. multilocularis and does not discuss the liver form of the disease (AE) will be excluded in this review. Sources will be excluded if they are not written in English. Since 2001, there has been a slow, but steady increase in AE human cases in Europe (Jenkins et al., 2025). In Canada, the index case of confirmed AE in dogs occurred in 2009, and the first confirmed human case (since 1928) was identified in 2013 (James & Boyd, 1937; Massolo et al., 2014; Peregrine et al., 2012). Due to the upward trend of AE prevalence since the early 2000s (in Europe and North America), resources prior to 2000 will be excluded. At the stage of the full-text screening, literature will be included only if exposures, risk factors, predictors, and determinants are discussed and relevant to AE. Exposure risk factors will be defined as external factors that increase the likelihood of a human or animal being exposed to E. multilocularis which could lead to AE. Host risk factors will be defined as internal factors that affect an organism’s predisposition to AE. Data from sources that meet these criteria will be extracted into an Excel spreadsheet for analysis and synthesis. The results will highlight risk factors of consensus across research and grey literature worldwide to address the gaps in knowledge of AE transmission in North America and Europe.