• We describe a patient with Crouzon syndrome and congenital tracheal stenosis. The trachea lacked rings and was completely cartilaginous, making it rigid and narrow. The respiratory difficulties that are present in patients with Crouzon syndrome have been ascribed to the nasal or choanal defects that are associated with this condition. However, the presence of congenital tracheobronchial abnormalities as a cause of respiratory problems has not been widely investigated. Congenital tracheal stenosis may occur as an isolated defect or as one of many other congenital defects. In patients with Crouzon syndrome who have recurrent respiratory problems, congenital tracheal or bronchial defects should be considered since surgical intervention may correct the defect.