Congenital tracheomalacia is the most common congenital tracheal abnormality and occurs in approximately 1:2,100 children. Tracheomalacia can be isolated or associated with other airway anomalies such as laryngomalacia, bronchomalacia, and large laryngeal clefts. Also, an abnormal division of the embryonic foregut is frequently associated with congenital tracheomalacia; mostly in the form of proximal esophageal atresia with distal tracheoesophageal fistula. In such cases, the ratio between the cartilage ring and the posterior membranous wall drops from the normal ratio of 4 to 5:1 to 2 to 3:1. The diagnosis can be made upon clinical history and physical examination as well as by pulmonary function testing, computed tomography, dynamic magnetic resonance imaging, and fiberoptic bronchoscopy. Other approaches such as tracheobronchography have been used, but have not been validated for the diagnosis of tracheomalacia; fluoroscopy can be specific for diagnosing tracheomalacia, but lacks a reasonable sensitivity. Tracheomalacia is often self-limited and will resolve or become asymptomatic by the second year of life without intervention. For patients who remain symptomatic, possible treatments include pharmacotherapy, positive pressure application, and surgery.