Congenital dilatation of the bile doer is an uncommon lesion requiring
surgical treatment. Complete excision of the diseased bile duct preve
nts development of carcinoma of the bile duct. We report three cases o
f congenital dilation of the binary three, each illustrating a particu
lar aspect of the therapeutic strategy. One patient had an unusual ano
maly of the pancreaticobiliary junction that required pancreaticoduode
nectomy. Another patient developed carcinoma of the bile duct 15 years
after an interval derivation of a choledocal cyst intrahepatic bile d
ucts associated with congenital hepatic fibrosis.