Pulmonary atresia with intact ventricular septum (PAIVS) is rare, less than 1% of congenital heart disease. It needs a therapeutic approach according to its individual morphologic feature. Surgical treatment of valvotomy and modified Blalock-Taussig shunt or non-surgical interventional catheter balloon valvuloplasty can be used for mild to moderate hypoplasia of right ventricle. Fontan operation can be considered for less optimum morphological substrate of two ventricular repair. A 3- day-old male neonate was admitted with cyanosis and cardiac murmur. On echocardiogram, he had membranous pulmonary atresia with intact ventricular septum, normal sized tripartite right ventricle, large atrial septal defect with right-to-left shunt, small sized patent ductus arteriosus, and moderate tricuspid regurgitation. He was treated with intravenous continuous infusion of prostaglandin E1 (PGE1) at once. On the third day of hospitalization, Balloon valvuloplasty was performed. After insertion of patent ductus arteriosus stent on the tenth day, PGE1 infusion was discontinued. On the fifteenth day, he was discharged. Now, he is 9 months old and has nearly normal cardiac structure and function with 97% of percutaneous oxygen saturation.