Objective: The aim of this systematic review and meta-analysis was to provide a comprehensive estimate for spontaneous coronary artery dissection (SCAD) related mortality, and explore factors associated with an increased risk of death. Background: SCAD is an infrequent but increasingly recognized cause of acute coronary syndrome. Despite a growing body of evidence, there have been few detailed examinations of SCAD associated mortality. Methods: We searched MEDLINE, EMBASE, Cochrane, Web of Science and Google Scholar databases through May 7, 2020. We included studies reporting mortality data, confirmed SCAD with coronary angiography and included ≥10 participants. We excluded non-English studies, conference abstracts, review articles and duplicate datasets. Random-effects meta-analysis and meta-regression were used to evaluate estimates and predictors of mortality. Results: From an initial 1,131 articles, 34 studies with 2,817 patients were eligible for inclusion. The weighted mean age was 50 years, and 84% of participants were female. The pooled estimate for SCAD mortality was 1% (Proportion 0.01; 95% CI, 0.00–0.02). The mean duration of follow-up was 33 months. Meta-regression showed male sex was associated with 3.5-fold increased odds of mortality (OR, 3.50; 95% CI, 1.22–10.03). In addition, smoking (current or previous) was associated with a 15-fold increased risk of mortality (OR 15.32; 95% CI, 2.88–81.41). Conclusions: This meta-analysis has shown that SCAD is associated with favorable survival outcomes with an estimated mortality of 1% over a mean follow-up period of 33 months. We also found male sex and smoking were associated with an increased risk of mortality.