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Peer-reviewed articles (n = 124) examining associations between medical mistrust (MM) and health outcomes from four databases, between January 1998 and May 2018, were reviewed; 36 qualitative and 88 quantitative studies met the inclusion criteria. The Williams and Mohammed framework guided our narrative synthesis of the studies; it argues that basic causes (e.g., biased institutions) affect the social status of marginalized groups which in turn effects multiple proximal pathways leading to responses and poor health. Most studies were cross-sectional with US-based samples. The MM in qualitative studies were categorized as interpersonal (n = 30), systemic (n = 22), and/or vicarious (n = 18); 25% did not explicitly note the basic causes of MM and race/ethnicity was often confounded with socioeconomic status (SES). All but three studies discussed an association between MM and a behavior response; no study focused on an actual health outcome. Most quantitative studies used multivariate regression analyses; only 15 of the 88 utilized advanced modeling techniques (e.g., mediation). Most (75%) studies did not describe basic causes for MM and 43% utilized low income samples. MM was conceptualized as a predictor/proximal pathway (in 73 studies) associated with a variety of responses, most commonly behavioral (e.g., diminished adherence); 14 studies found an association between MM and a specific health measure. This review underscores the need for future qualitative studies to place MM central to their research questions as in-depth descriptions of MM were limited. Future quantitative studies should replicate findings using more advanced analytical strategies that examine the relationship between MM and health outcomes.