BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is being identified with increasing regularity and may be associated with myocardial ischemia and/or sudden death. There is an on-going controversy regarding which patients should have medical treatment, which should undergo surgical treatment, and what are the optimal surgical techniques to achieve lasting success. METHODS: This was a retrospective review of 230 patients who underwent surgical repair of AAOCA at a single institution. Median age at surgery was 17 years. 130 had pre-operative symptoms prompting their referral for surgery including with sudden death. An additional 32 patients had associated congenital heart defects. 29% were asymptomatic. 187 had an anomalous right coronary whereas 43 had an anomalous left coronary. 192 had an intramural course while 38 did not including 13 with an intraconal course. RESULTS: 86 patients underwent an unroofing procedure, 123 underwent coronary reimplantation, 13 had repair of their intraconal left main, and 8 had other procedures. There were no early or late deaths with a median follow-up of 4 years. Six patients (2.6%) have required re-operations for recurrent symptoms and/or ischemia. Three of the six had anatomic narrowing of the coronary artery and the other three had an undiagnosed myocardial bridge. Five re-operations (5.8%) were in the unroofing cohort while 0 were in the reimplantation group. CONCLUSIONS: Surgical repair of AAOCA can be performed with extremely low mortality and low incidence of re-operation. Our current preference is to perform coronary reimplantation as this mitigates some pitfalls of the unroofing procedure.