We reported a case of non-Hodgkin’s lymphoma whereliver involvement was the predominant clinicalmanifestation.A 27-year old man presented wiht markedlyelevated serum aspartate aminotrasferase,alanineaminotransferase and lactate dehydrogenase,reducedprothrombin activity,thrombocytopenic purpura andhepato-splenomegaly without adenopathy.Viral,toxic,autoimmune and metabolic liver diseases were excluded.Bone marrow biopsy showed an intracapillary infiltrationof T-lymphocytes with no evidence of lipid storage disease.Because of a progressive spleen enlargement,splenectomywas performed.Histological examination showedlymphomatous intrasinuses invasion of the spleen.Immunohistochemical investigation revealed the Tphenotype of the neoplastic cells:CD45+,CD45RO+,CD3+,CD4-,CD8-,TIAl-.About 50 % of the lymphoidcells expressed CD56 antigen.The diagnosis ofhepatosplenic T cell lymphoma was done.The patient wastreated with chemotherapy,which induced a completeremission.Eighteen months later,he had a first relapsewith increased aspartate aminotransferase,alanineaminotransferase,lactate dehydrogenase,thrombocytopenic purpura and blast in the peripheral blood.In spite of autologous bone marrow transplantation,hedied twenty months after the diagnosis.Even in the absenceof a mass lesion or lymphoadenopathy,hepatosplenic T-cell lymphoma should be considered in the differentialdiagnosis of a patient whose clinical course is atypical foracute hepatic dysfunction. We reported a case of non-Hodgkin’s lymphoma whereliver involvement was the predominant clinical manifestation. A 27-year old man presented wiht markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic, autoimmune and metabolic liver diseases were excluded.Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease.Because of a progressive spleen enlargement, splenectomywas performed. Histological examination showinglymphomatous intrasinuses invasion of the spleen. Immunohistochemical investigation revealed the Tphenotype of the neoplastic cells: CD45 +, CD45RO +, CD3 +, CD4-, CD8-, TIA1-. About 50% of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatopathy T cell lymphoma was done. the patient wastreated with chemotherapy, which induced a complete remission.Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, thrombocytopenic purpura and blast in the peripheral blood. In spite of autologous bone marrow transplantation, hedied twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic T-cell lymphoma should be considered in the differentialdiagnosis of a patient whose clinical course is atypical foracute hepatic dysfunction.