Congenital malformations of the trachea include a variety of conditions that cause respiratory distress in neonates and infants. A number of anomalies are self-limiting while others are life-threatening and require immediate therapy. The prevalence of congenital airway malformations has been estimated to range between 0.2 and 1 in 10,000 live births. The most frequent congenital tracheal malformations are: tracheomalacia, congenital tracheal stenosis, laryngotracheal cleft and tracheal agenesis. The management of congenital tracheal malformations is complex and requires an individualized approach delivered by a multidiscipilinary team within centralized units with the necessary expertise.