Tumors of the papilla of Vater are very rare. Despite advanced imaging tech
niques the distinction between benign and malignant tumors remains very dif
ficult. Because most ampullary and periampullary tumors are malignant, prim
ary management is surgical, Here we report the case of a 65-year-old man wi
th biliary obstruction caused by an ampullary hamartoma simulating cancer.
The correct diagnosis was not established until surgery.