Absent pulmonary valve (APV) syndrome is a rare anomaly that is usually associated with tetralogy of Fallot (TOF) and causes severe respiratory distress by compression of the trachea. Mortality following surgical repair in these patients is increased, especially in infants presenting with severe respiratory compromise. Preoperative ventilator dependency and age at operation are risk factors for mortality after surgical repair. Between 1995 and 1999, 5 patients underwent surgical treatment for TOF with APV. The mean age at operation was 9 months (range: 1 to 29 months), and the mean weight at operation was 5.2kg (3.6-9.1kg). Among these patients, 3 patients presented with severe respiratory distress caused by dilatation of aneurysmal pulmonary artery (pulmonary artery index>2,000) and 2 of these patients were dependent on a respirator prior to surgical treatment. Reduction of dilated pulmonary artery, including anterior wall resection and posterior placation, was performed in all patients. In the patients with severe preoperative respiratory compromise, the right ventricular outflow tract was reconstructed with an extracardiac conduit with autologous pericardial valve leaflets to avoid pulmonary valve regurgitation after the operation and a transannular patch with a PTFE valve was used in 2 patients without respiratory compromise. One patient died suddenly 7 months after surgical intervention. Three of the surviving patients underwent a second right ventricular outflow tract reconstruction because of progressive right ventricular outflow tract stenosis. In two of these patients the right ventricular outflow tract was reconstructed with an extracardiac conduit with autologous pericardial valve leaflets at first operation. All surviving patients are well without any physical limitations and have been placed in NYHA class I. Despite the need for reoperation for right ventricular outflow tract stenosis, aggressive surgical treatment for TOF with APV has produced a satisfactory mid-term outcome.