Progress has been made in the treatment of children born with hypoplastic left heart syndrome. From a mortality of more than 95% at 1 month of age in an era prior to surgical intervention, an actuarial survival of 58% at 5 years of age for staged surgical palliation is now being achieved. The short-term results with cardiac transplantation also appear to be excellent. Efforts are being directed at identifying potential risk factors, and fetal ultrasonography is capable of monitoring the progression of this malformation in utero. Refinements in surgical technique and postoperative care have been achieved, reducing the overall risk of the Norwood operation and sudden hemody-namic instability in the intensive care unit. Practice patterns and perceptions of outcome vary widely. Presently there is no unanimity of opinion that surgical therapy should be offered to all patients, and comfort care continues to be a family option.