IntroductionPrimary lymphoma of the prostate is very rare. In this paper we present a case of early stage non-Hodgkin lymphoma of the prostate managed with six cycles of rituximab-based chemotherapy, and review the related literature.Case presentationAn 84-year-old man was admitted to our hospital having signs and symptoms suggestive of prostatic disease for 3 years. Histological and immunocytochemical studies of trans-urethral biopsy of the prostate showed diffuse large B-cell lymphoma. Radiological assessment of disease confirmed the diagnosis of early stage lymphoma of the prostate. The patient was managed by 6 of rituximab 375 mg/m2 on day 1, cyclophosphamide 750 mg/m2 on day 1, doxorubicin 50 mg/m2 on day 1, vincristine 1.4 mg/m2 on day 1, and prednisone 50 mg/m2 on days 1 to 5 with complete clinical and radiological response. He remained disease free, until now, 30 months after the end of chemotherapy.ConclusionAccording to the literature, the treatment and prognosis of primary lymphoma of the prostate is the same as that of other nodal lymphomas. Rituximab-based regimen should be considered in the management of prostatic diffuse large B-cell lymphoma.