Absent pulmonary valve syndrome is represented by rudimentary nodules without identifiable leaflets, and it is commonly associated with a ventricular septal defect and tetralogy of Fallot. Unilateral absence of a pulmonary artery is also a rare congenital anomaly. The following is a case report of a one-day-old newborn with cyanosis. The physical examination revealed a to-and-fro murmur and no expansion of the left hemithorax. Echocardiography revealed tetralogy of Fallot, absent pulmonary valve, and enlarged main and right pulmonary arteries. The catheterization and angiography confirmed the diagnosis and absence of the left pulmonary artery.