Eight cases of pulmonary atresia with intact ventricular septum, are reviewed. Three corresponded to the group with small or hypoplastic right ventricle and five to the one with normal or enlarged right ventricular cavity. The electrocardiogram was of less value than plain chest radiography for the differential diagnosis of both groups. Cardiac catheterization revealed a right to left atrial shunt and the suprasystemic level of the right ventricular pressure. Selective angiocardiography demonstrates the stop of contrast at the level of pulmonary valve. Five patients were operated. An ascending aorta to right pulmonary artery anastomosis, Waterston type, was performed in three patients. Pulmonary valvotomy was carried out in the others.