Laryngotracheoesophageal clefts (LTECs) are rare congenital defects of variable severity depending on the extent of malformation. Management of a complete LTEC represents a major surgical and anesthetic challenge. The main problems are achieving adequate operative exposure and maintaining ventilatory support during and after the operation. We describe correction of a type IV LTEC extending down to the carina in an infant who had respiratory distress at birth. Surgical repair was achieved in a single stage by an anterior sternotomy approach on the 11th day of life. The procedure was facilitated by cardiopulmonary bypass. After the operation, the infant was intubated, mechanically ventilated, and sedated. Nissen fundoplication and gastrostomy were carried out on the 11th postoperative day. The child was extubated on the 12th postoperative day. The rationale for this method and its advantages in comparison with other surgical approaches are discussed.