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Objective — Performing global population-based studies and comparing the results with other studies can reveal regional differences in the distribution of HLA-B27 in patients with Behcet disease. The aim of study was reviewing the distribution of HLA-B27 and Behcet disease in the world. Material and Methods — A systematic review search was conducted of the MeSH keywords of Behcet disease, HLA-B27 and B27 in ProQuest, Scopus, EMBASE, SID, IranDoc and IranMedex from 1975 to 2017 in Iran. Data was analyzed using meta-analysis (random effect model) in Software CMA2. The heterogeneity of studies was assessed with I2index. Results — The pooled frequency of HLA-B27 in patients with patients throughout the world for 11,483 samples and 36 studies was 7.4%. A higher frequency of HLA-B27 was found in Europe for, followed by the Middle East (7.2 (4.5-11.7)%, P<0.001) and Far East (6.4 (2.1-19.4)%, P<0.001). By country, the highest frequency was found for Great Britain 16.7% (8.6-33.1), followed by Iran 7.2% (4.5-11.7) and Turkey 5.9 (3.6-9.9)%. Conclusion — The results demonstrate the role of HLA–B genes such as -B27 on Behcet disease and provide information about the causes and effects of the subject to help design health programs and carry out future investigations on the odds ratio.