Three infants presenting with respiratory distress required early ventilator support. With attempts at extubation recurrent airway obstruction occurred. The clinical course was marked by recurrent episodes of hyperinflation, atelectasis, and pneumonia. Bronchoscopy, bronchography, and chest fluoroscopy revealed extensive collapse of the trachea and main stem bronchi. Two of the infants had gastroesophageal reflux and recurrent aspiration. Treatment of tracheobronchomalacia (TBM) was carried out with a tracheostomy tube attached to a portable CPAP apparatus. Initially CPAP was maintained at 10 cm of water and subsequently weaning was achieved by gradual decreasing of both positive pressure and hours of treatment per day. Total treatment time ranged from 13 to 25 months. Feedings were carried out via gastrostomy. Two infants with severe gastroesophageal reflux underwent fundoplication. Each infant was successfully weaned from distending pressure and decanulated. The treatment of severe TBM with long-term CPAP appears to be a reasonable alternative or adjunct to surgical procedures such as tracheopexy, resection, external splinting and tracheobronchoplasty.