Pulmonary atresia with intact ventricular septum is a extremely rare congenital cardiac anomaly. With the history of cyanosis and failure to thrive, this anomaly should not be excluded and emergency management is necessary. Our patient was 69 day-old male with pulmonary atresia which was confirmed by cardiac angiography. Prostaglandin E1 was used for maintenance of pulmonary blood flow preoperatively. Right ventricular outflow reconstruction with pericardial patch and concomitant pulmonary valvotomy were done on beating heart for palliation. With this method, growing of right ventricle and tricuspid annulus are highly expected.