Background: Patients with aortic aortic arch obstruction and complex cardiac anomalies are a surgical challenge with significant mortality. For these patients, we have adopted an aggressive approach of single-stage repair with excellent results. Methods: Since 2000, 14 patients with aortic arch obstruction and complex cardiac anomalies have undergone complete single-stage repair. As a diagnosis, there were Taussing-Bing anomaly in 7, transposition of great arteries with ventricular septal defect(VSD) in 2, Truncus arteriosus with VSD in 1, aortopulmonary window in 1, double outlet right ventricle with doubly committed VSD in 1, Shone complex in 1, supramitral ring in 1. The median age of repair was 12.5 days and the median weight was 3.28kg. End-to-side or extended end-to-end anastomosis was used for the repair of arch anomaly. In the recent two patients with severe great artery size discrepancy, we used small pieces of main pulmonary artery for the arch repair. Results: There was one early death in the patient with Shone complex (parachute mitral valve, monocusp aortic valve, coarctation of aorta) (1/14, 7.1%). He underwent aortic valve commissurotomy and papillary muscle splitting with coarctation repair. He died from severe left ventricular failure. The median follow-up is 21.5 months. There was no reoperation for recurrent artic arch obstruction, but one patient needed balloon angioplasty (1/13, 7.7%). All survivors are currently well from a cardiac point of view. Conclusions: Concomitant single-stage repair for aortic arch obstruction with complex cardiac anomalies achieves excellent survival and can be the surgical procedure of choice.