Familial Hypercholesterolemia (FH) is an inherited disorder leading to increased risk of premature atherosclerotic cardiovascular disease. This risk can be ameliorated through adherence to pharmacological treatment and salient lifestyle behaviors (e.g., physical activity participation, healthy eating). Identifying theory-based, modifiable determinants of these behaviors may inform behavioral interventions promoting participation in FH self-management behaviors. We aimed to identify the belief-based social cognition constructs uniquely associated with intentions to perform, and actual participation in, FH self-management behaviors in the extant research. A systematic database search identified studies (k = 9, N = 1394) reporting relations between social cognition theory constructs and intention toward, or actual participation in, self-management behaviors in FH patients. As no studies examining prospectively-measured behaviors were identified, we tested relations among social cognition constructs, intentions, and past FH-self-management behavior using random effects multi-level meta-analysis and meta-analytic structural equation modelling. We found non-zero averaged correlations among the key social cognition constructs (attitudes, norms, risk perceptions, self-efficacy), intentions, and past behavior. A meta-analytic structural equation model indicated non-zero averaged direct effects of attitudes, norms, self-efficacy, and past behavior on FH self-management behavioral intentions. There were also non-zero averaged indirect effects of past behavior on intentions mediated by the social cognition constructs. Findings provide evidence to support the proposed model and highlight the importance of personal, normative, and capacity related beliefs and past experience as unique correlates of intentions to perform FH self-management behaviors. The model may signal potential constructs that could be targeted in behavioral interventions to promote participation in FH self-management behaviors. •Social cognition correlates of self-management intentions in familial hypercholesterolemia (FH) patients were meta-analyzed.•Studies reporting correlations among social cognition constructs and FH self-management behavioral intentions were identified.•Meta-Analysis identified attitudes, norms, and self-efficacy as key correlates of intentions.•Associations between risk perceptions and self-management behavioral intentions was no different from zero.•Findings may inform development of interventions to promote FH patients' self-management behaviors.