Midaortic syndrome is an uncommon disease characterized by segmental or diffuse narrowing of abdominal or distal descending thorasic aorta. Majority of the cases are idiopathic but some are secondary to fibromuscular dysplasia, giant cell arteritis, Moyamoya disease or genetic diseases such as neurofibromatosis, Williams' syndrome and Alagille syndrome. It is very rare in newborn period. Here we present a 40 days old infant who admitted to hospital for respiratory distress, hypertension, heart failure and was diagnosed as midaortic syndrome. She had medical treatment but unfortunately died due to heart failure when she was two months old age.