Adenocarcinoma of ampulla of Vater causing obstructive jaundice in an 11-year-old boy is reported. He first underwent pylorus-preserving duodenectomy, which later necessitated classical Whipple operation because of the microscopical evidence of the disease beyond surgical margins. This child is the youngest case of adenocarcinoma of ampulla of Vater in the English-language literature. Copyright (C) 1997 by W.B. Saunders Company.