A 29-year-old Japanese male was diagnosed as having autoimmune hemolytic anemia (AIHA) in 1985, and systemic lupus erythematosus (SLE) in 1988. In 1989 a large mass was found of the right posterior area in the liver by computed tomography and ultrasonography. The liver biopsy specimen showed non-Hodgkin lymphoma, diffuse large cell type. No adenopathies or other extranodal involvements were detected. He was treated with five courses of CHOP, followed by involved field irradiation 24 Gy. On completion of chemoradiotherapy, the mass was smaller in size with a sign of partial necrosis. Gallium scan was negative suggesting the achievement of a complete remission. Primary hepatic lymphoma is extremely rare, and its occurrence in patients with AIHA and SLE has not been reported previously. We also review the previously reported cases of primary hepatic lymphoma with respect to its clinical management and abscess the therapeutic strategy of this unusual extranodal lymphoma.