This report concerns a rare case of follicular lymphoma with features suggestive of primary hepatic lymphoma. At the disease onset, multiple nodular lesions in the liver and small para-aortic nodes were detected by abdominal magnetic resonance imaging without generalized lymphadenopathy. After careful observation for three months, lymphadenopathy was observed in the right occipital, para-aortic, and bilateral inguinal regions. The patient was treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) and achieved complete remission for more than 2 years. To the best of our knowledge, this is the ninth report of primary hepatic follicular lymphoma. Insufficient cases have been reported to determine the long-term outcome and clinical characteristics of primary hepatic follicular lymphoma. However, it seems that patients with primary hepatic follicular lymphoma that are treated with appropriate chemotherapy with or without surgical resection have favorable outcomes.