Background: Primary splenic lymphoma is a rare disease with heterogeneous definition, compromising less than 1% of non-Hodgkin's lymphoma. Case presentation: We are reporting a case of 50 year old man who presented splenomegaly, fever, weight loss and night sweats. The investigations revealed a splenomegaly with splenic lesion which cancorrespond to abscess. The patient underwent splenectomy for definitive diagnosis and treatment. Diagnosis of primary diffuse large B cell lymphoma of the spleen was made and confirmed by immunochemistry and the patient received 6 cycles of RCHOP (Rituximab + cyclophosphamide + Adriamycin + vincristine + prednisone) and he is currently well and in complete remission more than 1 year after diagnosis. Conclusion: Primary splenic lymphoma is rare; splenectomy is an important step in its diagnosis and treatment. Early splenectomy combined with chemotherapy is an optimal strategy.