Congenital tracheal stenosis (CTS) is a rare but potentially life-threatening disease which results in congnital airway lesion. CTS is often associated with cardiovascular anomalies and presented with a wide spectrum of symptoms. CTS has challenged pediatric surgeons for decades. Various classic approaches and new techniques, including computational fluid dynamics, tissue-engineering trachea, and 3D printing have been proposed for diagnosis and treatment of CTS. This review provides a snapshot of the main progress of diagnosis and treatment of CTS.