Although atherosclerosis remains the major cause of acute coronary syndrome, there are many other etiologies that should be taken into account, especially in young patients with no atherosclerotic risk factors. Coronary involvement is extremely rare in patients with Behçet's disease, notably in young patients. In addition, acute inferior myocardial infarction revealing Behçet's disease has rarely been reported. Through this article, we report a case of Behçet's disease with arterial involvement diagnosed after myocardial infarction resulting from thrombosis of the right coronary artery in a 50-year-old woman with no specific medical history.