Tracheal agenesis is a rare congenital anomaly and typically has fatal consequences. Associated congenital malformations are present in 90 per cent of cases, most frequently affecting the cardiovascular or gastrointestinal systems and the genitourinary tract. Affected infants lack prenatal symptoms and usually present with severe respiratory distress, absence of audible crying and difficult or impossible endotracheal intubation, leading to failed airway management and irreversible cerebral hypoxia. The authors report an infant with tracheal agenesis who presented with respiratory failure after birth. The clinical features, embryology and classification schemes are presented in the hope of increasing awareness, thus making earlier diagnosis possible and thereby improving survival.