Anomalous aortic origin of the coronary arteries is a rare defect and is associated with an increased risk of cardiac symptoms including sudden death. The most important subtypes are those in which the anomalous coronary artery arises from the wrong sinus as in anomalous left main coronary artery from the right sinus and anomalous right coronary artery from the left sinus. Ischemia can result when the flap-like orifice becomes obstructed, usually during exercise. Indications for surgical intervention are evolving. Currently, the favored approach for repair is an unroofing type of procedure that results in relocation of the coronary artery into the appropriate sinus of Valsalva.