Patients with schizophrenia have a higher incidence of coronary artery disease. This meta-analysis was performed to evaluate the influence of a prior diagnosis of schizophrenia on mortality following acute coronary syndrome (ACS). Relevant longitudinal follow-up studies were obtained via systematic search of PubMed and Embase databases. A random effect model was used to perform the meta-analysis. This meta-analysis included 3,611,343 hospitalized patients with ACS from nine follow-up studies. The results show that, in patients with schizophrenia, ACS was associated with a significantly higher risk of mortality (multivariate adjusted risk ratio [RR]: 1.66, p < .001) with significant heterogeneity (I2 = 93%) compared to the results of mentally healthy patients. Subgroup analyses demonstrated that patients with schizophrenia were associated with higher ACS mortality within one month (RR: 1.68, p < .001) and during a follow-up period of >= one year (RR: 1.71, p = .01), in studies with (RR: 1.65, p = .06) and without the adjustment of revascularization treatments (RR: 1.68, p = .004), as compared with the results of mentally healthy patients. These results indicate that patients with schizophrenia have a higher than expected mortality risk in the case of acute coronary events.