Tracheomalacia with anterior great vessel compression is a common disorder in infants and children, which can lead to life-threatening airway occlusion. In this study, a large number of patients underwent anterior aortopexy to provide a more normal distal airway. Thirty-two infants and children with tracheomalacia associated with esophageal atresia-tracheoesophageal fistula (18), vascular ring (8), abnormal innominate artery takeoff (4), and primary (2) were evaluated with bronchoscopy, magnetic resonance imaging, and pulmonary functions. Aortopexy was accomplished through left thoracotomy and suture fixation of the aorta and innominate artery to the posterior sternum. Intraoperative bronchoscopy showed marked improvement in airway caliber and rigidity, and no patients had further obstructive episodes. Forced expiratory volume improved from 52% +/- 4% of predicted to 82% +/- 3%. Aortopexy is a simple procedure for the treatment of distal tracheomalacia that is immediately effective and provides permanent relief of obstructive episodes.