A 13-year-old boy with partial anomalous pulmonary venous connection (PAPVC) having an intact atrial septum (IAS) is reported. He had open heart surgery 3 years ago at another hospital, but the cardiac septum was intact and PDA was ligated. After his discharge, cardiomegaly and increased right pulmonary vasculature were seen in chest roentgenograms. Our cardiac catheterization data and pulmonary angiograms showed that he had a PAPVC with IAS. Surgical correction was accomplished by utilizing a polytetrafluoroethylene graft with external ring support and interposing it between the right upper pulmonary vein and the left atrium during thoracotomy without the need for extracorporeal circulation. The postoperative course was uneventful and he was discharged on the 14th postoperative day. At 3 months postoperatively, radionuclide studies demonstrated no evidence of pulmonary congestion or graft obstruction. To our knowledge, this is the first clinical report of prosthetic replacement of the pulmonary venous pathway with graft patency demonstrated postoperatively.