A 27-year-old woman complained of a severe stridor caused by a right-sided double aortic arch with a right-sided descending thoracic aorta. A smaller left-sided aortic arch had an atretic segment located between the left common carotid artery and an aortic diverticulum from which the left subclavian artery originated. Through a left fourth thoracotomy, the atretic segment, which caused a compression of the trachea and esophagus, was ligated and divided. The ligamentum arteriosum could not be identified on that side. After the operation she was completely relieved of her symptoms.