A surgical correction was successfully performed in a adult female who had pulmonary atresia with almost non-confluent pulmonary artery, PDA and ventricular septal defect. Initially as a first stage of corrective surgery isolation of patent ductus arteriosus, ligation of aortopulmonary collaterals and identification of the pathologic anatomy of left pulmonary artery through left posterolateral approach were performed. At the second stage, a week after the initial operation, total correction was done making both pulmonary arteries confluent with albumin coated woven-dacron graft, external valved conduit and closure of large VSD of subarterial and perimembranous type. The PDA was ligated with previously encircled heavy stitch soon after partial bypass was started. Although massive bleeding from anastomotic site of dilated left pulmonary artery to the graft occurred preoperatively, postoperative functional improvement was excellent in terms of disappearance of cyanosis and normal exercise tolerance.