Primary splenic lymphoma is uncommon, constituting only 1-2% of all patients with malignant lymphoma. Despite the rarity of this malignancy, the number of primary splenic lymphoma being reported has increased due to its ambiguous definition used in the literature. We describe a case of a 41-year-old man with chronic hepatitis C virus infection presenting abdominal discomfort in the upper left quadrant, weakness, nausea and vomiting. Abdominal computed tomography revealed nodules in the congested splenic parenchyma. Splenectomy was performed and an analysis of the spleen diagnosed B-cell non-Hodgkin's lymphoma. Biopsy of the liver showed evidence of hepatitis C virus. Bone marrow biopsy revealed no tumor infiltration. The patient has been followed to date, has progressed quite well and remains essentially asymptomatic. Recently, an etiologically important role has been suggested for hepatitis C virus infection in the development of B-cell non Hodgkin's lymphoma. Lymphotropism of hepatitis C virus may play a pathological role in the development of non Hodgkin's lymphoma. It is important to add lymphoma to the list of differential diagnosis of extrahepatic disorders in patients with chronic hepatitis C virus infection.