Vanishing bile duct syndrome is a rare, acquired disease that has been described in different pathologic conditions' including adverse drug reactions, autoimmune diseases, graft vs host disease, and neoplasms. It is a condition characterized by progressive loss of intrahepatic bile ducts leading to ductopenia and cholestasis. Here we report a 27-year-old female who presented with jaundice and cholestatic hepatitis and was finally diagnosed with vanishing Bile duct syndrome secondary to Hodgkin lymphoma. Physicians need to consider a range of differential diagnoses, especially malignancies, in suspected cases of vanishing bile duct syndrome.