Airway malformations such as laryngeal atresia, tracheal agenesis and subglottic stenosis are rare and present at birth with significant respiratory distress with or without stridor. There may be an initial improvement on bag and mask ventilation. Repeated attempts at intubation are met with failure. The related embryology and clinical aspect of airway malformations have been discussed. The prognosis in tracheal agenesis is universally fatal but cases with laryngeal atresia and subglottic stenosis may be saved with prompt tracheostomy and later surgical reconstruction.