Asphyxiating thoracic dystrophy is a rare, complex malformation with a broad spectrum of clinical expression. Surgery is indicated only in severe cases in which failure to intervene will result in progressive pulmonary damage and eventual death. Conventional surgical techniques for expanding the thoracic cage diameter by sternotomy and the insertion of a metal prosthesis for anterior chest wall stability usually provide these patients with the time needed for thoracic cage growth. However, some of the most severe cases may require a two-stage approach. Hence, management should be directed toward resolving immediate ventilatory problems and minimizing secondary damage to the lungs caused by prolonged ventilatory support.