Objective: To describe an unusual case of acute liver failure due to Hodgkin's lymphoma. Case Presentation and Intervention: A 37-year-old man was admitted with jaundice and abdominal distension. Physical examination showed tender hepatosplenomegaly, ascites, grade I encephalopathy, left cervical (2 x 1 cm) and axillary (1 x 1 cm) lymph nodes. The laboratory data revealed elevated serum bilirubin, transaminases, lactate dehydrogenase, and coagulation defects. Initially, primary liver disease was considered, but a liver biopsy revealed infiltration of the liver by Hodgkin's lymphoma that was confirmed by lymph node biopsy. Hodgkin's lymphoma was of lymphocyte depletion type. Conclusion: This case demonstrates that in the presence of lymphadenopathy involving acute liver failure, hematological malignancies should be taken into consideration. Liver and lymph node biopsies should be performed as early as possible. Copyright (C) 2004 S. Karger AG, Basel.