Coronary artery anomalies are a frequently neglected topic in cardiology. However, they are an important cause of cardiac ischemia and sudden death. Anomalous pulmonary artery and anomalous aortic origins of the coronaries are 2 major types of coronary artery anomalies. This article will examine the occurrence of these anomalies in the absence of associated congenital defects. A review of the literature reveals that the anomaly most commonly associated with sudden death is a left main coronary artery arising from the right aortic sinus of Valsalva with an interarterial course. The increased risk of sudden death with this anomaly has been associated with any of 4 high-risk features: slit-like ostium, acute-angle take-off, intramural aortic segment, and interarterial course.