Two patients with congenital tracheal stenosis, one who had segmental type stenosis and the other who had extensive type stenosis, were surgically treated at our hospital. The patient with segmental tracheal stenosis was successfully treated by resection and anastomosis. Five years postoperatively, although she was asymptomatic, endoscopic examination revealed a circumferential stricture with an inner diameter of about 3 mm in the upper trachea. A second operation was thus performed to remove the stenotic trachea and her postoperative course was uneventful. The second patient was a male whose extensive tracheal stenosis was associated with vascular ring and tracheal bronchus. Division of the vascular ring was not effective and a tracheoplasty using costal cartilage was performed twice, also unsuccessfully. He is now managed with a tracheostomy, using a long endotracheal tube which was specially designed for him. A recent bronchoscopy revealed a flaccid distal trachea. In this report, we also discuss the technical problems associated with treating congenital tracheal stenosis.