BACKGROUND Complete correction of atrioventricular septal defect (AVSD) associated with tetralogy of Fallot (TOF) has been reported to account for an increased surgical risk. Impaired right ventricular function after classic transventricular repair, residual outflow tract stenosis, and incompetence of the pulmonary or atrioventricular valves are considered to be essential factors affecting the results. METHODS From 3/95 to 6/98 six consecutive patients with AVSD and TOF underwent repair (age 18 months to 7.3 years) using a combined transatrial-transpulmonary approach. RV outflow tract balloon dilatation preceded transatrial correction in 4 patients. Pulmonary annulotomy but not transanular patching was necessary in 4 cases. The septal defects were closed by two separate patches using a Dacron patch with short depth and anterior extension for the ventricular component. RESULTS All patients survived and had stable sinus rhythm. Echocardiography demonstrated mild, but hemodynamically insignificant mitral regurgitation in two and tricuspid regurgitation in four patients. Right ventricle to pulmonary artery gradients ranged from 5 to 35 mmHg (mean 24.2 mmHg) without progression. During follow-up ranging from 4 months to 3.5 years (mean 16.8 months) no reoperation was necessary. CONCLUSIONS The transatrial-transpulmonary approach for correction of AVSD with TOF contributes to improved results after repair of this rare combination of defects.