The management of a critical airway in infants and toddlers with congenital tracheal stenosis (CTS) continues to be an enormous challenge to the surgeon. Until recently, this condition often proved fatal. Improvements in surgical techniques, anesthetic management, and postoperative critical care have resulted in successful outcomes in children not long ago considered untreatable. However, issues such as the best operative approach and the optimal perioperative management are still unresolved. The diagnosis of CTS, often delayed, must be considered in any infant with stridor, wheezing, cyanosis, or recurrent episodes of pneumonia. Associated anomalies are the rule, including frequently vascular rings and rarely pulmonary agenesis. These defects can be repaired with conventional ventilatory support under cardiopulmonary bypass, or using extracorporeal membrane oxygenation (ECMO). We report our experience in which ECMO was used to support two patients with CTS during the perioperative period. ECMO proved to be both safe and practical, allowing unrushed, precise repair of the tracheal stenosis and providing brief postoperative support. Perioperative outcomes were excellent, although one of our patients died months after the repair. A review of the literature and our experience in which ECMO was used to provide cardiopulmonary support during repair of CTS showed uniformly successful perioperative outcomes.