Background: Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus traveling between the aorta and pulmonary artery is associated with ischemia and sudden death. Methods: A retrospective review of 36 patients (23 male) who underwent operation between October 1992 and August 2008 for AAOCA was performed. Median age was 47 years (range, 13 to 82 years). Angina, shortness of breath, or syncope was present in 29 (81%), and 9 of 21 (43%) had an abnormal stress test. Coronary or computed tomographic angiography demonstrated an anomalous left main coronary artery arising from the right sinus in 13 (36%), right coronary artery arising from the left sinus in 21 (58%), and left anterior descending artery arising from the right sinus traveling between the aorta and pulmonary artery in 2 (5%). An intramural course was identified on preoperative imaging in 34 (94%). Although no patients had significant associated atherosclerotic coronary artery disease, 5 (14%) had previous acute myocardial infarction related to the AAOCA. Results: Operation included coronary artery bypass grafting in 14 patients and unroofing in 22; 6 patients had associated cardiac procedures performed. There were no early deaths. There was one late death secondary to a subdural bleed. At follow-up (mean 1.1 years; maximum 14 years), chest pain recurred in 1 patient who had coronary artery bypass grafting. No recurrent symptoms were noted in the unroofing group. Conclusions: Unroofing of an anomalous coronary artery can be performed safely with excellent results in the majority of patients. When concomitant atherosclerotic coronary artery disease is present, coronary artery bypass grafting is an appropriate alternative. © 2009 The Society of Thoracic Surgeons.