OBJECTIVE The authors' experience in surgical management of tetralogy of Fallot in association with complete atrioventricular septal defect (CAVSD-TOF) is reported. METHODS Between January 1990 and September 2001, 146 children with CAVSD underwent complete correction. ASD closure was performed using a one-patch technique, with autologous pericardial patch. Seventeen patients presented with CAVSD-TOF, of whom nine (53%) had undergone previous palliation. Mean age at repair was 2.9 +/- 1.9 years. The mean gradient across the right ventricular outflow tract (RVOT) was 63 +/- 16 mmHg. Six patients (35%) required a transannular patch. RESULTS Overall in-hospital mortality was 10%. Among patients with CAVSD-TOF, three died in hospital (18%); causes of death were progressive heart failure (n = 2) and multiple organ failure (n = 1). Two patients required mediastinal exploration due to significant bleeding, and two late reoperation for severe left atrioventricular valve regurgitation after intracardiac repair. Mean follow up was 36 +/- 34 months. All patients survived and are currently in NYHA class I or II. At follow up, mean gradient across the RVOT was 17 +/- 6 mmHg, significantly less than the preoperative value (p < 0.001). CONCLUSIONS Complete repair in patients with CAVSD-TOF offers acceptable early and mid-term outcome in terms of mortality, morbidity and reoperation rate. Palliation prior to repair may be preferred in cases with small pulmonary arteries, or in severely cyanotic neonates. The RVOT should be managed as for isolated TOF, but a transatrial-transpulmonary approach is the surgery of choice.