Congenital vascular anomalies of the great vessels causing marked tracheoesophageal compression are rare. Notable symptoms usually manifest in the neonatal period and include stridor, repeated cyanotic attacks, recurrent pneumonia, and dysphagia. Although the diagnosis can often be made from barium swallow studies, the preoperative examination should nevertheless include an endoscopy. This is essential if the degree of tracheomalacia is to be adequately assessed and other tracheobronchial abnormalities are to be identified. Possible airway problems can then be anticipated, thus minimizing postoperative respiratory difficulties.