BACKGROUND The association of tetralogy of Fallot with total anomalous pulmonary venous drainage (TAPVD) is rare. We report our experience with this condition and review the literature. METHODS Between January 1997 and May 2008, 6 patients (aged 3 months to 5 years; median weight, 10 kg) with combined tetralogy of Fallot with TAPVD underwent complete primary repair at All India Institute of Medical Sciences, New Delhi, India. Their records were retrospectively reviewed. RESULTS A correct preoperative diagnosis was available in 5 patients by echocardiography and cardiac catheterization. Four patients had supracardiac TAPVD, and 1 each had TAPVD to the coronary sinus and right atrium. There were no early or late deaths. Median follow-up was 37.5 months +/- 46.8 months (range, 2 to 112). All patients are in New York Heart Association class I. Follow-up echocardiograms have revealed no significant abnormalities and have documented normal biventricular function and pulmonary artery pressures. One patient underwent a 24-hour Holter examination at 68 months of follow-up for investigation of a new-onset 2:1 atrioventricular block, for which a permanent pacemaker implantation is planned. CONCLUSIONS When diagnosed accurately, complete primary repair is possible in patients with tetralogy of Fallot and TAPVD and the outcomes are satisfactory with planned surgery.