Abstract： Objective To analyze the surgical strategy and result of one-stage repair for congenital aortic arch disease associated with other cardiac anomalies. Methods Between April 1993 and November 2009, 25 consecutive patients aged 26 d to 6. 5 years underwent one-stage repair for congenital aortic arch disease with other cardiac anomalies. Among them, 6 patients had coarctation of aorta, 6 patients had interrupted aortic arch, and 13 cases had hypoplasia of aortic arch. The surgical techniques include excision of the anterior wall of pulmonary artery, resection of patent ductus arteriosus tissue, aortic arch reconstruction with autologous pulmonary artery wall, reconstruction of the pulmonary artery and repair of the associated defects. Results Twenty-four patients survived and recovered uneventfully. One patient died of pulmonary hypertention crisis in hospital. The reconstruction of the aorta and the correction of the intracardiac anomalies were proved by postoperative echocardiography and CT scan. There were no neurological or other complications. The follow-up showed that all patients developed normally and there were no restenosis of the aorta arch. Conclusions With the benefits of growth potential and less tension, autologous pulmonary artery tissue is an optimal choice in aortic arch reconstruction. One-stage repair of congenital aortic arch disease associated with other cardiac anomalies can achieve good results.