A 2-month old male was admitted due to repeated cyanotic attacks. He had suffered from stridor and retractive breathing since birth. Double aortic arch was diagnosed and the vascular ring formed by the double aortic arch was compressing the trachea. Multirow detector computed tomography showed that he had a right-dominant double aortic arch with left ductus arteriosus and an aberrant left subclavian artery, and that the narrowest part of the trachea, where the diameter was 2.0 mm, was located 9.0 mm above the carina. Airway management in patients with extreme narrowing of the trachea is challenging for anesthesiologists. He was scheduled for ligation and division of the left aortic arch and ductus arteriosus. In the operating theater, anesthesia was slowly induced with sevoflurane (0-4%) in oxygen. After mask ventilation was confirmed to be adequate, a 4.0 mm internal diameter endotracheal tube (ETT) was inserted and advanced smoothly beyond the tracheal stenosis. The tip of the ETT was placed just above the carina using a fiber optic bronchoscope (fiberscope) that was passed through the ETT. Since mechanical ventilation was adequate, vecuronium was administered. Surgery was conducted in the right lateral position and using a left thoracotomy approach. Anesthesia was maintained with sevoflurane (2-3%). After positioning, right one-lung ventilation was performed unexpectedly. However, anesthetic management was achieved without difficult ventilation during surgery. The tip of the ETT was pulled past the stenotic part before transfer to the intensive care unit (ICU). A patent trachea during spontaneous breathing under CPAP (5 and 2 cmH(2)O) was confirmed with a bronchofiberscope in the ICU. After weaning from mechanical ventilation, he had the persistence of mild stridor despite improvement of respiratory symptoms.