Tracheal agenesis is a rare congenital anomaly. We report a case and review the cases previously reported. Clinical features that might indicate tracheal agenesis include antenatal polyhydramnios, severe respiratory distress, absence of an audible cry, failure to advance an endotracheal tube beyond the larynx, a palpable distal trachea, clinical improvement after esophageal intubation, and roentgenographic absence of a tracheal air column with an abnormal position of the carina. For immediate management of the affected infant, we recommend intubation of the esophagus with an endotracheal tube to provide an air passage, and determination of the level of the defect by careful use of contrast material and roentgenography. Infants having type I tracheal agenesis may benefit from immediate tracheostomy.