Introduction: The purpose of this study was to examine whether the insertion (I) or deletion (D) of angiotensinconverting enzyme (ACE) polymorphism confers susceptibility to sarcoidosis. Methods: A meta-analysis on the associations between the ACE I/D polymorphism and sarcoidosis was conducted. Results: Seventeen comparison studies consisting of 1556 cases and 2381 controls were available for the meta-analysis. Meta-analysis revealed a significant association between the D allele and sarcoidosis (OR 1.206, 95% CI 1.049-1.386, p = 0.009). An ethnicity-specific meta-analysis of the DD+ID genotype showed an association with sarcoidosis in East Asians (OR 1.342, 95% CI 1.041-1.729, p = 0.023). An association was found between the DD genotype of the ACE I/D polymorphism and sarcoidosis (OR 1.251, 95% CI 1.070-1.463, p = 0.005). Furthermore, stratification by ethnicity indicated an association between the DD genotype and sarcoidosis in Europeans (OR = 1.215, 95% CI = 1.010-1.462, p = 0.039). Conclusions: Our meta-analysis demonstrates that the ACE I/D polymorphism is associated with susceptibility to sarcoidosis in European and East Asian populations.