Behçet disease is a chronic, inflammatory multisystemic condition of unknown etiology. Cardiac involvement, known as cardio-Behçet disease is seen in approximately 5% of patients with Behçet disease. Surgical approach to cardio-Behçet patients is still a debate and the results of surgery are not satisfactory. The purpose of this paper is to review the published literature world-wide, concerning the cardiac operations of patients with cardio- Behçet disease and discuss the proposed surgical approaches for the treatment. Eight surgically treated cardio- Behçet patients with coronary stenosis and aneurysm were found in the literature. Fourteen authors reported forty nine Behçet patients to have valvular operations. Forty four of these patients underwent reoperations and eleven of them were lost. In especially young patients presenting with coronary aneurysms, acute occlusions or unexplained aortic regurgitations Behçet disease should always be suspected and kept in mind to be investigated among the other causes of the presenting symptoms. Surgery should be avoided during the acute exacerbation period of the disease, if possible. Prompt immunosuppressive treatment and close follow up should accompany the cardiac operation. Arterial punctures for coronary angiography, blood gases or surgery performed to bypass an aneurysm may provoke development of further aneurysms at the site of vascular puncture or anastomosis and prosthetic heart valve detachment constitutes a serious threat for the patients with valve replacement.