Background: Behçet’s disease is a vasculitis, seen more frequently around the Mediterranean and the Far East, and evinces with oral and genital ulcerations, skin lesions and uveitis. Carbonic anhydrase (CA) is a metalloenzyme which is widely distributed in the living world, and it is essential for the regulation of acid-base balance. Anti-CA antibodies have been reported in many disorders, such as systemic lupus erythematosus, Sjögren’s syndrome, rheumatoid arthritis, endometriosis, idiopathic chronic pancreatitis, type 1 diabetes and Graves’ disease. The goal of this study was to investigate CA I and II autoantibodies in Behçet's disease (BD). Methods: 35 patients with BD and 29 healthy controls were included in the study and CA I and II autoantibody levels were investigated by ELISA. Results: The CA I and II autoantibody levels of BD group were significantly higher than the healthy group (p=0.013, p inf 0.0001, respectively). A cut-off value of 0.250 ABSU for anti-CA I was associated with 34 % sensitivity and 100 % specificity and a cut-off value of 0.171 ABSU for anti-CA II was associated with 54 % sensitivity and 100 % specificity for predicting BD. Conclusion: The CA I and II autoantibody levels in patients with BD were found higher compared to control group and the results suggest that CA I and II autoantibodies may be involved in the pathogenesis of BD.