Primary renal non-Hodgkin's lymphoma (NHL) with acute renal failure is a very rare condition, which frequently occurs in bilateral renal involvement. We report a 26-year-old male with primary bilateral renal NHL presenting with acute renal failure. A CT scan of the abdomen showed markedly enlarged kidneys with multinodularity and para-aortic lymphadenopathy. A percutaneous renal biopsy demonstrated 'follicular center lymphoma, diffuse, small cell'. Thirteen cycles of systemic chemotherapy with cyclophosphamide-doxorubicin-vincristine-prednisolone (CHOP) and ifosfamide-methotrexate-etoposide-bleomycin (IMVP-Bleo) regimens were administered, which resulted in normalization of renal function with improvement of renal lymphoma. Since there was no further change of renal lesions after initial partial remission, a follow-up renal biopsy was performed 10 months after diagnosis, and no residual lymphoma was found.