Behcet's disease is a multisystemic, rheumatic disorder characterized by oral and genital ulceration and ocular inflammation. Although cardiac involvement is not rare as a manifestation of Behcet's disease, coronary arteritis is reported seldom. We present the first case of left main coronary artery thrombosis in literature leading to acute myocardial infarction that was successfully treated with thrombolytic therapy and glycoprotein IIb/IIIa receptor inhibitors in Behcet's disease.