Congenital tracheal stenosis is a rare congenital anomaly, with less than 70 reported cases in the literature. The presenting signs and symptoms of stridor, recurrent pneumonia, and respiratory distress are commonly seen in other conditions. The rarity of congenital tracheal stenosis and the diverse presentations make accurate early diagnosis difficult and frequently lead to inappropriate treatment. We treated three patients with congenital tracheal stenosis who presented with different sites of stenosis. Each patient displayed different symptoms and required individualized management. The treatment of congenital tracheal stenosis depends on identifying the site and extent of the stenosis. We reviewed the embryogenesis and treatment of this abnormality and developed a new classification system that will aid in the management of congenital tracheal stenosis.