Congenital stenosis involving the entire length of the trachea has generally been regarded as a fatal disease. Tracheoplasty using costal cartilage grafts to enlarge the lumen was successfully employed in such a case, and the technique is described. A 12-mo-old female was referred with recurrent severe respiratory distress since birth. By tracheoscopy and bronchography, the entire trachea was seen to be stenotic. The left bronchus was of normal caliber by bronchogram and the left lung was over inflated, while the right lung was aplastic. Through a midsternal thoracotomy, the left bronchus was incised and cannulated for ventilation. Longitudinal incision of the entire length of the anterior wall of the trachea permitted the advance of a nasotracheal tube along the inner surface of the divided trachea to the carina. Two pieces of costal cartilage were used to fill the defect in the anterior wall of the trachea. The grafts were attached to the tracheal edges by interrupted 5-0 Dexon sutures. The endotracheal tube was successfully removed two months later. The subsequent course of the patient has been satisfactory.