Objective To evaluate the outcome of surgical repair of interrupted aortic arch (IAA) combined with anomalies. Methods We retrospectively analyzed the clinical data of 48 patients with IAA combined with anomalies undergoing one-stage biventricular repair in Shanghai Children's Medical Center from November 2006 to April 2016. There were 25 males and 23 females with a median age of 29 d (range, 8 to 91 d) and a mean weight of 3.80±0.67 kg. All patients underwent end-to-end anastomosis with patch augmentation, and relief of left ventricular outflow tract obstruction (LVOTO) was performed in 11 patients. Results In IAA children with anomalies, 39 (81.3%) suffered noncomplex lesions and 9 (18.8%) complex lesions. Mean follow-up was 72.1±19.7 months for 38 patients. There were 6 in-hospital deaths and 3 patients died during follow-up. The early survival rate was 87.5%, 5-year rate 83.3% and 10-year rate 81.3%. Reintervention was required in 10 patients, including 8 with subsequent LVOTO and 2 with anastomotic stenosis. Conclusion End-to-end anastomosis with patch augmentation is effective for IAA.