Behçet's disease is a systemic vasculitic syndrome with unknown etiology. The aim of the present study was to compare the Helicobacter pylori seropositivity and cytotoxin associated gene-A status in patients with Behçet's disease. Ninety-one patients with Behçet's disease and 83 age- and sex-matched persons with or without any gastrointestinal complaints were included in the study. Demographic characteristics and H. pylori IgG, IgM, and cytotoxin-associated gene-A IgG status of the Behçet's disease and the control groups were analyzed. The influence of eradication therapy on clinical findings was also determined. The prevalence of H. pylori IgG seropositivity was slightly but not significantly higher in patients with Behçet's disease compared to the controls [72 (79.1%) vs 56 (67.5%), (p = 0.082)]. The prevalence of cytotoxin-associated gene A positivity was significantly higher in Behçet's disease compared to the controls [59 (64.8.%) vs 32 (38.5%), respectively, (p = 0.002)]. Eradication of H. pylori has significantly decreased clinical manifestations such as oral and genital ulceration, arthritis/arthralgia, and cutaneous findings of Bahçet's disease. Our study indicates that H. pylori may be involved in the pathogenesis of Behçet's disease or disease activity might be enhanced due to induced inflammation or altered immunity.