OBJECTIVES We reviewed our experience with repair of congenital coronary artery fistulas. METHODS From June 1983 to December 2009, 46 patients (median age, 59 years; range, 1-84 years) underwent surgical repair. The presenting symptoms included angina in 16 patients (35%), congestive heart failure in 11 (24%), and bacterial endocarditis in 5 (11%). Preoperatively, 9 patients (20%) had at least moderate tricuspid regurgitation. Coronary artery dominance was right in 38 patients (83%). Coronary artery aneurysms were found in 8 patients (17%). The most common pattern was right coronary artery-to-coronary sinus fistula (18 patients, 39%); 11 patients had (23%) more than 1 fistula. One patient had undergone previous coil embolization. RESULTS Cardiopulmonary bypass was used in 39 patients (85%), with extracardiac and intracardiac repair performed in 30 (65%) and 16 (35%), respectively. The most common associated procedures were coronary artery bypass in 13 patients (28%). Early mortality occurred in 1 patient (2%). Postoperative myocardial infarction occurred in 5 patients (11%); 4 of these patients underwent simple ligation or division of their fistulas. The mean follow-up was 6 ± 5.8 years (maximum, 22 years). Late mortality occurred in 11 patients (24%). Two patients underwent reoperation for severe tricuspid regurgitation. Survival was significantly reduced compared with the age- and gender-matched population (P = .03). Residual fistulas were detected in 3 patients (6%), with no reintervention needed. CONCLUSIONS Perioperative myocardial infarction is an important complication of ligation of coronary artery fistulas and can contribute to reduced late survival. The tricuspid valve should be evaluated carefully at repair because of the relatively high rate of residual regurgitation in survivors.