Uveal melanoma (UM) is one of the most primary intraocular tumors. Patients with a family history and susceptibility of cancer are more likely to develop UM. Many of gene mutations like BAP1 and GNAQ and GNA11 are implicated in this disease. Gene BARD1 (BRCA1-associated Ring domain protein-1), located on 2q34-35, plays vital roles in the aforementioned cellular molecular mechanisms. The Bard1 protein makes a stable heterodimeric complex with BRCA1. This heterodimeric complex exhibits significant E3 ubiquitin ligase activity as well as can independently induce p-53 dependent apoptosis pathway, thus any inhibiting mutation in this gene may lead to UM. The BARD1gene polymorphism rs2229571 evokes the heterodimer ubiquitin ligase activity, contributing to cell cycle regulation, transcription regulation, and DNA repair. Our study investigated the prevalence of BARD1 gene polymorphism rs2229571 in Russian population and its contribution to developing UM in patients from Central Russia. Genomic DNA was extracted from peripheral blood of 42 patients with UM as a case group, 23 individuals with benign choroidal nevus as a risk group and 61 healthy individuals as a control group, and population group of 100 randomly selected Russian volunteers. Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) was used for amplification. DNA fragments were separated by 2% agarose gel electrophoresis and visualized by ethidium bromide and UV. The Chi- square test was used for estimation of distribution of genotypes and alleles and the significant differences between the studied groups. Our results exhibited a significantly higher frequency of GG homozygotes in the case group (21.4%) when compared with the control (1.6%, p=0.0001). Frequency of the GG genotype was found also higher in the risk group than its frequency in the control however did not achieve the significant level (13.0%, p=0.091). These results reflect that the development of UM and progressive choroidal nevus is associated with the homozygous GG genotype in patients (OR=13.025; 95% CI 2.964-57.242). Based on the population analysis, the genotype frequencies of BARD1 gene polymorphism rs2229571 are CC 42%, GC 47%, GG 11%, and alleles frequencies C 65.5%, G 34.5%, which is consistent with the results of our case-control study. Our findings of the population are in large consistence with the frequencies reported in NCBI; East Asian (C 65.87%, G 34.13%) and African population (C 62.10%, G 37.90), whereas disagreeing with the frequencies in European population (C 35.88%, G 64.12%). The BARD1 rs2229571 polymorphism is recommended as a predictive genetic biomarker for choroidal neoplasms