CD5 positive diffuse large B cell lymphoma is a very rare subtype of diffuse large B cell lymphoma with an aggressive lymphoid neoplasm. Extranodal involvement of lymphoma usually appears secondarily in the setting of disseminated disease, but the disease may originate from various extralymphatic organs. We present a case of primary extranodal lymphoma with coexistence of skin and renal involvement with rapidly progressive renal failure due to lymhpomatous infiltration. Renal biopsy revealed diffuse infiltration of the renal parenchyma by CD20+ atypical lymphoid cells. The patient was treated with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone. After the chemotherapy regimen, a dramatic improvement in the clinical picture was noticed.