A-23-year-old man visited our clinic with upper airway symptoms. Bilateral expiratory wheezes were detected on physical examination. The enlargement of the right pulmonary artery and lower bifurcation of the trachea observed on chest X-ray led us to perform chest computed tomography (CT). This revealed a narrowed trachea and left pulmonary artery malformation (pulmonary artery sling). A three-dimensional (3D) CT and a virtual bronchoscopy were performed on clinical suspicion of anatomical abnormality. The image showed concentric rings at the site of the tracheal narrowing (complete tracheal rings); we therefore made a diagnosis of congenital tracheal stenosis (CTS). Complete tracheal rings and pulmonary artery sling are typical findings in CTS. From 1980 to 2017, only 11 cases of CTS diagnosed in the adult phase were reported (comprising 9 females and 2 males, age at diagnosis 16 to 57 years). The median minimum tracheal diameter was 6.6mm, and no cases underwent tracheoplasty. Recently, cases diagnosed by 3D CT have been increasingly reported. Since abnormalities of the airway tract cause respiratory distress, it is extremely rare to survive the neonatal and infant phases without severe airway problems. Here, we report a case of CTS diagnosed in the adult phase and review the recent literature.