Pulmonary artery sling is an uncommon vascular anomaly and can be life threatening when it causes tracheal compression. We report on a 14-day-old boy who presented with respiratory distress soon after birth. A series of examinations showed tracheal stenosis due to a pulmonary artery sling. Surgery was performed with the aid of cardiopulmonary bypass. The external compression and intrisic stenosis could not be resolved by vascular surgery because of tracheal malacia and a complete tracheal ring. We recommend cutting extra holes 1 to 2 cm from the distal end of the endotracheal tube for endobronchial intubation. The airway obstruction was resolved successfully with a custom-made endobronchial tube. However, the patient died of pneumomediastinum and pneumothorax induced by barotrauma, on the fourth postoperative day.