Congenital long-segment tracheal stenosis which involves nearly entire trachea and carina is very rare disease, but leads to life threatening obstruction in infancy and childhood. Symptoms are ranged from stridor and wheezing to severe cyanosis and respiratory failure. Routine chest X-ray is somewhat helpful to diagnose it, but definitive diagnosis can be made by bronchoscopy or tracheogram for severely narrowed tracheal lumen.Recently, we experienced a case of congenital tracheal stenois, type 1 by Cantrell classification with carinal involvement. After costal cartilage was designed as oval shaped flap and covered with pericardium, anterior and posterior augmentation was done with prepared costal cartilage.This patient died of respiratory failure at 13 days postoperatively, probably due to sustaining obstruction in association in with failure to make a sufficient widening at carinal level.Important issues in the management of congenital tracheal stenosis are rapid diagnosis, selection of appropriate surgical procedure, and detailed anesthetic schedule.In the future, more biocompatible material and more effective surgical procedures should be studied to reduce the surgical mortality and morbidity of the complicated tracheal stenosis.