Improved understanding of the postoperative physiology and experience with the surgical techniques and perioperative care of patients with hypoplastic left heart syndrome have resulted in improved outcomes. Over the past few years, numerous modifications to the intraoperative and postoperative management of these patients have been described. It is likely that in combination, these modifications and better understanding of the unique physiology after the Norwood procedure are responsible for decreasing early mortality. This review describes and discusses the current surgical and medical management of patients undergoing first-stage palliation for hypoplastic left heart syndrome and its variants.