Congenital tracheo-oesophageal fistula in the adult is rare, and there have only been 16 such reported cases in the English literature. The concomitant presence of a cancer of the oesophago-gastric junction however has not been previously reported, and presents a treatment dilemma as to whether a staged or simultaneous surgical treatment should be performed. We report such a case that was successfully treated by staged surgical therapy.