Double aortic arch (DAA) is a rare vascular congenital abnormality. Since a vascular ring surrounds bronchus and esophagus, any oral or nasal intubation can physically cause fatal aortoesophageal fistula (AEF). We report herein the first case of association of DAA and superior mesenteric artery (SMA) syndrome and the second case of AEF caused by nasogastric intubation in an adult with DAA. A 19-year-old woman visited our hospital for nausea and vomiting. She was diagnosed with SMA syndrome by computed tomography (CT). Nasogastric intubation relieved her symptoms in 4 days. Extramural compression with top ulceration was found in esophagogastroduodenoscopy on the 5th hospital day. She suddenly showed massive hematemesis on the 12th hospital day. AEF was found by CT. Soon, she died despite of intensive care. Retrospective interview disclosed the fact that DAA was pointed out in her childhood. We conclude that intubation must be avoided in DAA and a detailed clinical interview about DAA is mandatory to avoid AEF.