Hypoplastic left heart syndrome is a congenital heart defect where only right ventricle remains functionally unimpaired. Soon after birth, the newborns become severely ill with cardiorespiratory collapse which is followed quickly by death without immediate medical intervention. A crucial step in treatment is staged surgical palliation. Infants with hypoplastic left heart syndrome must be managed by a multidisciplinary team. A five year survival rate is 70% and is still increasing due to the improvement of surgical techniques used to treat this cardiac abnormality, the development of pediatric intensive care and the improvement of postoperative care.