Background: Hepatic localization of non Hodgkin's lymphoma is generally secondary. Primary localizations are rare.
Aim: To report a rare case of primary hepatic lymphoma particular by its association with dermatopolymyositis.
Observation: A 55-year-old woman with a past medical history of dermatopolymyositis diagnosed one year before presented with abdominal pain and fever. Laboratory tests showed pancytopenia. Radiologic examination revealed multiple hepatic masses. Surgical biopsy revealed a large B cell hepatic lymphoma. No other localizations were found so the diagnosis of primary hepatic lymphoma was retained. The patient died after a few days due to a severe sepsis.
Conclusion: Primary hepatic lymphoma is a rare tumor with a bad prognosis. Its diagnosis is based on histologic examination. Treatment of these tumors remains non consensual.