We report the case of a 68-year-old man with adenomyoma of the distal common bile duct, fortuitously discovered on a surgical specimen of a Whipple procedure. To our knowledge, three similar cases have been reported; all patients were operated on with the diagnosis of malignancy: adenomyoma was always discovered on the surgical specimen. Adenomyomas are rare tumors and their origin is discussed. Adenomyoma of the distal common hile duct should be considered as enteropancreatic heterotopia.