Congenital tracheal stenosis is a rare but underdiagnosed anomaly which can present as life-threatening respiratory insufficiency in neonates and infants. Initial control of the airway is mandatory. Surgical correction is the mainstay of therapy and is achieved with low mortality. The type and extent of repair depends largely on the length of stenosis. Cardiac anomalies are frequently associated and may be addressed at the time of tracheal surgery. Despite initial satisfactory results, post-operative morbidity due to persistent granulation tissue is substantial. It is through a multidisciplinary approach and close follow-up of the repaired airway that these demanding patients are best cared for. The long-term quality of life remains uncertain.