Primary Hepatic Lymphoma (PHL) is rare and possibly associated with viral hepatitis and autoimmune diseases. Scleroderma could exceptionally be complicated by lymphoma. We describe PHL occurring in a 52-year-old female suffering scleroderma for eight years, with no history of cytotoxic or high-dose glucocorticoid therapy. CT scan, performed to work-up abdominal discomfort, constipation, and elevated alkaline phosphatase, showed a liver mass. Following left hepatic lobectomy, diffuse large B-cell lymphoma was diagnosed by pathological evaluations. Shortly after operation, chemotherapy began. The patient is alive and free of disease eight years after diagnosis of primary hepatic lymphoma. To the best of our knowledge, this is the first case of Primary Hepatic Lymphoma occurring in the setting of long-standing scleroderma. The fact that our patient had no history of immunosuppressive/ high-dose glucocorticoid therapy may indicate that similar immunologic abnormalities have pathogenetic role in both scleroderma and nonHodgkin’s lymphoma.