Diffuse, severe, macroscopically recognizable dilatation and hyperplas
ia of the peribiliary glands of intrahepatic and extrahepatic bile duc
ts is an extremely rare condition. To our knowledge, this is the first
such case reported in the literature. We encountered this condition i
n a patient with massive hepatic necrosis. The pathogenesis of the con
dition is discussed. Unfamiliarity with this pathology could lead to a
n erroneous diagnosis of a well-differentiated cholangiocarcinoma.