A 28-year-old man with Behçet's disease was presented with cardiac symptoms in addition to previous complaints of oral and genital ulcers. A diagnosis of thrombosis was made and patient began to receive anticoagulant and immunosuppressive therapy and was followed by echocardiographic examination. Despite medical therapy, thrombosis recurred. Surgical excision was performed and histological findings were consistent with organizing thrombus. Nature of cardiac involvement and review of literature on cardiac thrombosis in Behçet's disease was discussed.