ObjectivesTo systematically review the association between the SIX6 gene rs10483727 mutation and primary open angle glaucoma (POAG).MethodsPubMed, Web of Science, The Cochrane Library, CNKI, WanFang Data and VIP databases were searched to collect case-control studies on the SIX6 gene rs10483727 polymorphism and primary open angle glaucoma from inception to December 28th, 2018. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies. Meta-analysis was performed by Stata 12.0 software.ResultsSeventeen case-control studies in 16 papers were included, involving 9 886 patients and 19 663 controls. The results of meta-analysis showed that rs10483727 polymorphism in SIX6 gene was associated with the risk of POAG in the Asians and Caucasians. However, no association was found in the Africans.ConclusionsThe current evidence shows that rs10483727 polymorphism in SIX6 gene is associated with the risk of POAG in the Asians and Caucasians.