Pulmonary atresia with intact ventricular septum, and with VSD were uncommon congenital anomalies with high mortality in the neonatal period. Those survivals depend on an adequate interatrial communication or interventricular communication and pulmonary flow via large aortopulmonary collateral including PDA. Recently we experienced surgical correction of 2 cases pulmonary atresia with intact ventricular septum and with VSD. On case 1, 10-years old male patient was confirmed as pulmonary valvular membranous atresia with intact ventricular septum combined with large functioning PFO and mild tricuspid incompetence. So we performed total correction under the E.C.C, that was PDA ligation, RVOT reconstruction with monocusp valved outflow patch [16mm], repair of tricuspid insufficiency and closure of PFO. Post-operative hemodynamic result was good and there was no event during hospital course. On case 2, 16-years old female patient was diagnosed as pulmonary atresia with VSD and PDA. MPA was absent, remained fibrous cord like remnant and type of VSD was subaortic defect [3cm by 3cm in the size]. PDA was located at the usual site. Under the E.C.C. VSD patch closure through the right ventriculotomy, anastomosis between the right ventricular outflow tract and the pulmonary bifurcated site with the extra-cardiac Hancock valved conduit [22cm] and PDA ligation were performed.