Absent pulmonary valve syndrome with intact ventricular septum(APVS with IVS) is a rare congenital anomaly. The severe form of this syndrome, characterized by severe respiratory distress presented soon after birth, has been attributed to the compression of the airways caused by aneurysmal dilatation of pulmonary artery. Several operative treatments such as pulmonary valve insertion, or reductive angioplasty of pulmonary artery have been applied. We present a 3-day-old male who showed improvement after PDA ligation, reductive angioplasty of pulmonary artery, pulmonary valve reconstruction, and ASD closure.