Coronary artery anomalies are inevitably encountered during the performance of coronary arteriography. Proper identification by the angiographer is essential for conducting thorough and efficient investigations of the coronary artery system. Although most coronary artery anomalies are clinically insignificant, some are associated with symptoms and potentially serious complications. Further evaluation with stress testing and thallium scanning may be required. The cardiac surgeon should always be appraised preoperatively of the presence of coronary artery anomalies in order to avoid transsection, ligation, or iatrogenic occlusion of the vessel. Surgical repair of hemodynamically significant coronary artery anomalies has been performed with good results.