PURPOSE: The optimal surgical approach for aortic arch anomaly associated complex intracardiac defects is not universally agreed upon. We reviewed our recent experience of one-stage repair for the aortic arch anomaly associated with complex intracardiac defects. METHODS: We retrospectively analyzed the medical records of 15 consecutive patients with arch anomaly associated with complex intracardiac defects underwent going a one-stage repair through median sternotomy between September 2000 and May 2004. Transposition of the great artery or Taussig-Bing was associated in 9 patients, truncus arteriosus in 2, Shone' complex in 2, aorticopulmonary window in 1, and double outlet right ventricle in 1 patient. Aortic arch anomalies were coartation in 9 patients and interruption in 6. Age at operation ranged from 3 days to 23.4 months (median 12.5 days) and body weight ranged from 2.3 to 10.3 kg (mean 3.7+/-1.9 kg). RESULTS: There were 2 early deaths (13.3%) in the patients with Taussig-Bing anomaly and Shone's complex respectively. The causes of deaths were sepsis with right heart failure and pulmonary edema respectively. There were 2 late deaths. A patient with truncus arteriosus died suddenly of unknown cause and the other patient with Taussig-Bing anomaly died of ventricular dysfunction. One of 2 patients who died lately underwent balloon aortoplasty with success. There was no residual or recurrent coarctation in the rest of the patients. A patient required reoperation for left pulmonary artery stenosis. All survivors were in NYHA functional class I. CONCLUSION: One-stage repair of aortic arch with complex intracardiac defects can be performed with acceptable operative mortality. This procedure is strongly recommended as considering their grave prognosis and adverse effects of staged repair.