Abstract: Behçet's Syndrome (BS) is a variant vasculitis characterized by the recurrent oral and genital ulcers, skin lesions, uveitis, etc. The imbalance of Th cells and Treg cells and the abnormality of various cytokines play an important role in the pathogenesis of the disease. Nucleotide-binding oligomerization domin (NOD)-like receptor protein 3 (NLRP3) inflammasome is a vital component of the innate immune system, which can mediate activation of caspase-1 and promote the secretion of IL-1β and IL-18, thus participating in cell damage process. Abnormal activation of NLRP3 inflammasomes plays an important role in the occurrence and progression of various autoimmune diseases. Recently, researches on the correlation between NLRP3 inflammasomes and BS have received extensive attention. This review introduces the structure and function of NLRP3 inflammasomes and the research progress of NLRP3 inflammasomes in BS, aiming to provide new research ideas for the pathogenesis of BS.