Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm, with the majority (80–90% of cases) occurring in the upper aerodigestive tract. To our best knowledge, primary EMP from renal tissues is extremely rare. Herein, the diagnosis and treatment of a refractory primary EMP with renal involvement in a 53-year-old male patient is reported. The patient received radical nephrectomy followed by radiotherapy, and showed relapse 3 months after treatment. The cancer cells were sensitive to subsequent chemotherapy, however, the patient died of infection associated with the disease after almost 3.5 years following first presentation.