Abstract: Objective To understand the distribution of mutations in the Cytochrome P450 1B1 gene （CYP1B1） in patients with primary congenital glaucoma （PCG） in China. Methods A computerized literature search was carried out in PubMed, Web of Science, CNKI, Wanfang database, etc. to identify screening studies for CYP1B1 gene mutations in patients with primary congenital glaucoma in China. Systemized review was performed to assess the quality of the included studies and extract the data of studies in order to combine the rate of genetic mutation and acquire the mutation spectrum of the CYP1B1 gene in China patients with PCG. Results Nine studies meeting our criteria were identified and extracted. Forty-three distinct mutations were identified in 15.2%（95%CI 11.8%~19.0%）of all patients according to 9 scientific articles. It seems that there is no founder mutation in China, but also accumulates in some spots. Mutations 7990C>T （L385F）, 8006G>A（R390H） and 4124C>G（L107V） are common in China population. And that the mutation of L107V was unique to China people. Conclusion In China, the mutation rate in CYP1B1 gene in patient with PCG is lower. It suggests that there are other disease-causing genes in patient with PCG in China. The mutation spectrum of the CYP1B1 gene acquired may be useful to provide a reference for genetic mutation screening in patients with PCG.