Abstract: Objective To summarize the experience of surgical treatment of tetralogy of Fallot (TOF) with anomalous coronary artery. Methods From March 1993 to April 2006, 22 patients with TOF and anomalous coronary artery underwent repair. The resection of hypertrophied parietal, septal band and the ventricular septal defect (VSD) repairs were performed by trans-right ventricular outflow tract (RVOT) approach in 5 cases, and by transatrial approach in 17 cases, which consisted of 7 cases required a transannular patch to enlarge a pulmonary annulus, construction of a double barrel outlet in 6 cases, by autologous pericardium conduit (3 cases), homograft (1 case) and reflected anterior wall of the main pulmonary artery in combination with bovine pericardium (2 cases). Results There was one operative death because of the anomalous coronary artery impairment. The accessory left anterior descending artery was severed because it was mistaken for the conal arteryin 1 case, which caused failure to wean from bypass, after the left internal mammary artery was anastomosed to the accessory left anterior descending artery, the cardiopulmonary bypass (CPB) was stopped successfully. Mean early gradient(ΔP) was 23.4mmHg and ΔP＞20mmHg in 9 cases. Eighteen cases were followed up, mean time was 13.2 months. Late ΔP＞20mmHg in 7 cases, and ΔP were less than 20mmHg in 11 cases. Conclusion The repair of TOF with anomalous coronary artery is more safe by using the transatrial approach. The surgical reconstruction of RVOT depends on the anatomic characteristic of anomalous coronary artery.