BACKGROUND: Evidence suggests that genetic variations of exon 1 of mannose-binding lectin 2 (MBL2) may contribute to tuberculosis (TB) risk. Many studies have investigated the association between MBL2 exon 1 polymorphisms (rs1800450, rs1800451, and rs5030737) and TB risk, but yielded inconclusive results. METHOD: We conducted this meta-analysis of 26 eligible case-control studies that included 7952 cases and 9328 controls to identify the strength of association. Odds ratio (OR) and 95% CI were used to evaluate the strength of association. Statistical analyses were performed by using STATA 12.1. RESULTS: We found a statistically significant correlation between MBL2 exon 1 polymorphisms and increased TB risk among three models: allele model (O vs A: OR =1.18, 95% CI: 1.01-1.38, Pheterogeneity<0.0001, I2=85.8%), homozygote comparison (OO vs AA: OR =1.49, 95%CI: 1.02-2.18, Pheterogeneity<0.0001, I2=79.1%), dominant model (AO/OO vs AA: OR =1.20, 95% CI: 1.01-1.43, Pheterogeneity<0.0001, I2=83.5%), especially in studies based on Asian populations among five models: allele model (O vs A: OR =1.29, 95% CI: 1.11-1.51, Pheterogeneity<0.0001, I2=66.0%), homozygote comparison (OO vs AA: OR =1.67, 95% CI: 1.09-2.55, Pheterogeneity=0.008, I2=54.2%), heterozygote comparison (AO vs AA: OR =1.26, 95% CI: 1.05-1.50, Pheterogeneity=0.001, I2=62.9%), dominant model (AO/OO vs. AA: OR =1.31, 95% CI: 1.10-1.56, Pheterogeneity=0.001, I2=64.2%), and recessive model (OO vs AO/AA: OR =1.50, 95% CI: 1.01-2.22, Pheterogeneity=0.023, I2=48.0%). Meta-regression results revealed that source of controls (p=0.009), but not ethnicity (p=0.687), genotyping method (p=0.231), and sample size (p=0.451) contributed to the source of heterogeneity. CONCLUSION: This meta-analysis suggests that MBL2 exon 1 polymorphisms may contribute to TB risk, especially in Asian populations.