A six-day-old neonate was diagnosed with a severe form of the syndrome of absent pulmonary valve associated with pulmonary stenosis, aneurysmal dilatation of the pulmonary artery and rare findings including an intact ventricular septum and large ductus arteriosus. The patient underwent surgical repair by closed technique. Cardiac catheterization data, hemodynamic and clinical findings, and surgical technique are reported. Congenital absence of the pulmonary valve is a rare cardiac anomaly. An especially severe form occurs with ventricular septal defect and pulmonary stenosis. The usual findings are respiratory distress, aneurysmal dilatation of the pulmonary arteries and pulmonary stenosis.