Vanishing bile duct syndrome refers to a group of disorders which are characterized by prolonged cholestasis as a consequence of progressive destruction and disappearance of intrahepatic bile ducts. We present a case of Hodgkin's lymphoma presenting with vanishing bile duct syndrome. Liver damage is relatively common in Hodgkin's lymphoma. But only a small percentage of these patients develop jaundice. This may be secondary to drug toxicity, hemolysis, direct invasion by malignant cells or by extensive, obstructive lymphadenopathy. Vanishing bile duct syndrome secondary to Hodgkin's lymphoma is a rare cause of cholestasis in these patients. The mechanism of vanishing bile duct syndrome in Hodgkin's lymphoma is poorly explained, but a paraneoplastic effect seems most likely.