Purpose: The objective of this report is to evaluate our surgical approach to complete atrioventricular septal defect with tetralogy of Fallot (TOF) and to determine the best treatment for this malformation.Methods: From 2007 to 2012, 17 children underwent surgical correction of complete atrioventricular septal defect with TOF. Nine of these patients underwent complete correction, two underwent one and a half ventricle repair, and six underwent univentricular repair.Results: There was 1 in-hospital death and 1 late death. Patients with lower weight required longer intensive care unit stay (P <0.05; r = –0.834). Follow-up data were available for all patients who survived the operation (range, 3–60 months). The Kaplan-Meier curve indicated that 5-year survival was 88.2%. All survivors remained in good condition in New York Heart Association (NYHA) class I or II.Conclusion: Complete repair of atrioventricular septal defect with TOF has got favorable outcomes in terms of mortality and reoperation rate. Unbalanced atrioventricular septal defect with TOF can be managed well with one and a half ventricle repair. Two-patch technique through a transatrial approach alone is a good treatment choice for patients. Management of right ventricular outflow tract (RVOT) is similar to that of isolated TOF.