Pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries [abbreviated as PA+VSD+MAPCA in the following] has limited the success of attempts at accurate diagnosis and complete surgical repair. From April 1986 to September 1990, 23 patients with PA+VSD+MAPCA among 96 patients of PA+VSD in Seoul National University Children’s Hospital were encountered. The group comprised 14 male and 9 female patients with ages ranging from 17 days to 177 months [mean 49.6 months]. We operated one stage total repair on good pulmonary artery sized two patients by R.E.V. [Reparation a l’etage ventriculaire] and Rastelli operation respectively. And the 11 patients who had independent MAPCAs and hypoplastic central pulmonary artery were dealt with unifocalization and modified Blalock-Taussig Shunt and followed by second stage repair in 3 patients later. We successfully had managed 7 patients whose MAPCAs could be ligated with modified Blalock-Taussig Shunt and followed by second stage repair in 3 patients with R.E.V or Rastelli operation. Recently, three obstruction after 11 unifocalization procedures made us to try palliative right ventricle-pulmonary artery conduit operation by Gore-Tex vascular graft interposition under cardiopulmonary bypass. And so we managed another 3 patients with these procedures for the purpose of pulmonary artery growth whose central pulmonary artery were severely hypoplastic. We experienced one death after second stage repair whose central pulmonary artery was created by 12mm Gore-Tex vascular graft and was unifocalized.