<b><i>Background/Aims:</i></b> Several single nucleotide polymorphisms (SNPs) in the <i>PITX3 </i>gene have been associated with the risk for Parkinson's disease (PD). We conducted a systematic review and a meta-analysis including all the studies published on the risk of PD related with these polymorphisms. <b><i>Methods:</i></b> The systematic review was done using several databases. Eligible studies were included in the meta-analysis that was carried out using Meta-DiSc 1.1.1 software<i>.</i> Heterogeneity between studies was tested using the Q-statistic. <b><i>Results:</i></b> The meta-analysis included eight association studies for the <i>PITX3 </i>rs3758549 SNP (4,052 PD patients, 3,949 controls), eight studies for the <i>PITX3 </i>rs2281983 SNP (4,309 PD patients, 4,287 controls), and six studies for the rs4919621 SNP (2,724 PD patients, 2,285 controls), and the risk for PD, global diagnostic odds ratios (95% confidence intervals) for rs3758549, rs2281983, and rs4919621 were, respectively, 1.00 (0.89-1.12) (p = 0.979), 0.99 (0.91-1.09) (p = 0.896), and 0.98 (0.83-1.16) (p = 0.844) for the total group. The separate analysis in Caucasian and Chinese subjects on the frequency of the minor allele of the three SNPs analyzed did not show significant differences between PD patients and controls in both subgroups. rs2281983 and rs4919621 SNPs were related with early-onset PD risk in Caucasians. <b><i>Conclusion:</i></b> The results of the meta-analysis suggest that rs3758549, rs2281983, and rs4919621 SNPs are not major determinants of the risk for PD.