Tracheal stenosis is a rare, potentially life-threatening condition described as innate narrowing of the tracheal lumen. The causes of tracheal stenosis vary widely. The most common forms result from prolonged intubation, although congenital causes usually involve complete tracheal rings or compression from cardiovascular malformations. The condition historically has harbored a poor prognosis, but significant advances in radiologic diagnosis, cardiac bypass, and endoscopic and surgical treatments have led to a range of options, better overall survival, and reduced morbidity. The complex, long-term manifestations of tracheal stenosis point to the need for individualized treatment as well as multidisciplinary care.