Behçet's disease (BD) is a chronic systemic vasculitis that mainly characterized by recurrent oral ulcers, genital ulcers, uveitis and skin lesions. The pathogenesis of BD is still unknown. BD is considered to be an autoimmune disease triggered by infection or environmental factors in genetically susceptible individuals. Helper T cell 17 (Th17) play an important role in the pathogenesis of BD. Interferon regulatory factor 8 (IRF8) inhibits Th17 differentiation, thereby inhibiting the inflammation induced by Th17 and interleukin 17. Genomic studies suggest that IRF8-associated single nucleotide polymorphisms (SNPs) are risk loci of BD. In recent years, role of IRF8 inhibiting Th17 differentiation in the pathogenesis of BD has become a research focus.