Congenital bronchobiliary fistula (CBBF) is quite a rare malformation and the diagnosis is usually made within a few hours or years from birth because of lower respiratory diseases beginning from early infancy. Surgical repair is necessary. Of the 29 cases reported, 4 occurred in adults aged 22-32 years. We detected CBBF incidentally in a 65-year-old woman. During bronchoscopy and thoracic computed tomographic study of the pulmonary nodules, we found an accessory bronchus descending from the carina and composed of a dark green secretion that contained 10% bilirubin. Drip infusion cholangiography revealed air in the left bile duct. Cholescintigraphy showed dilatation of the left bile duct and radiotracer pooling at the top edge of the left hepatic lobe. These findings indicated a narrow fistula between the airway and biliary duct. We attributed the patient's long survival without major complications to the narrowness of the communication. To our best knowledge, this is the fifth and oldest reported adult diagnosed with CBBF.
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