Absent pulmonary valve is a rare congenital anomaly, characterized with a dysplastic or absent pulmonary valve tissue and severe pulmonary regurgitation. It is usually associated with tetralogy of Fallot (TOF). A 7-year-old girl with absent pulmonary valve, pulmonary artery an-eurysm and tetralogy of Fallot without cyanosis was op-erated and 10 months followed up result was reported.