Coronary artery aneurysm is a relatively rare disease, which may cause angina, myocardial infarction, or sudden unexpected death due to thrombosis, embolization or rupture. This report describes a case of a 46 year old male who suffered an inferior myocardial infarction with right ventricular involvement, third degree atrioventricular block, cardiogenic shock and late cardiac tamponade, all caused by a right coronary artery aneurysm. He was successfully treated with emergency coronary artery bypass grafting. A review of the literature is also given to emphasize the importance of prompt recognition and correct management of the coronary artery aneurysm.