A 39-year-old man who was known to have Behçet's syndrome suffered an acute posterior-wall myocardial infarction. The infarction occurred 13 years after the onset of the Behçet's disease, which had been marked by recurrent chorioretinitis and thrombosis of the retinal veins of both eyes. Coronary arteriography showed occlusion of the circumflex branch and an aneurysmal fistula between the left main branch and the pulmonary artery. The other coronary vessels were normal. A search for vascular risk factors revealed only cigarette smoking. Under a non-invasive treatment regimen, no complications of the mycoardial infarction were seen. During a 2-year medical follow-up, the patient was asymptomatic and did not show any further signs of Behçet's disease activity.