Congenital tracheobiliary fistulas are uncommon and almost always are diagnosed in the first weeks or months of life. Untreated congenital tracheobiliary fistulas cause intractable pneumonia and are revealed by a persistent cough. Medical therapy is not effective, and surgical repair is needed. A 22-year-old woman was treated successfully by resection of a fistula communicating between the tracheobronchial tree and the liver.