Objective  To explore the characteristics of clinical pathology, diagnosis, and prognosis of primary renal lymphoma (PRL).

  Methods  The clinical features, pathological features, immune phenotypes, treatment, and prognosis of 22 patients were retrospectively analyzed.

  Results  The PRL patients' ages ranged from 2 to 72 years (mean, 54.3 years), of which 13 patients were older than 50 years (59.1%). All of the 22 patients were diagnosed with non-Hodgkin's lymphoma (NHL), including 20 cases of B-cell lymphoma and 2 cases of T-cell lymphoma. Seven patients were still alive and survived for 6-50 months, but the other 15 were dead and survived for only 5-35 months.

  Conclusion  PRL is uncommon. Clinical manifestations and imaging performance specificity are not obvious. and easily misdiagnosed. Histopathology is still the golden standard for the final diagnosis of this entity. The kidney is most easily involved followed by the bladder. B-cell NHL is the common subtype, and the most common type is the diffuse large B-cell lymphoma. Up to now, no standard regime could be performed for PRL patients. At present, comprehensive therapy, including surgery and chemotherapy, is recommended. For patients with locally advanced or highly aggressive status, therapeutic effect with chemotherapy alone is usually satisfied.