Abstract: Objective To explore the clinical characteristics, treatment and outcomes of renal cell carcinoma （RCC） combined with phospholipase A2 receptor （PLA2R）-related membranous nephropathy （MN）. Methods The clinical and pathological data, diagnosis, treatment, and outcomes of three RCC patients with PLA2R-related MN were retrospectively analyzed. Relevant literatures were searched in Wanfang, VIP, CNKI, and PubMed databases using the following key words： “Renal Cell Carcinoma, Carcinoma, Renal Cell” and “phospholipase A2 Receptor, Receptors, Phospholipase A2” and “Membranous Nephropathy, Glomerulonephritis, Membranous”. Results Three RCC patients with PLA2R-related MN underwent partial or total tumor resection, with medications of short-term or long-term post-operative interferon or targeted drugs, hormone drugs and immunosuppressant or rituximab. They were followed up for 5-9 years. All the three cases were stable, with a complete remission of MN. After literature search, there were 17 cases of RCC combined with PLA2R-related MN at home and abroad, including 3 cases in this case report. The onset age of 20 patients ranged from 26 to 77 years old, with an average age of 60.75 years. Among them, 13 were male and 7 were female, and 10 cases were clearly diagnosed as ccRCC. Of the 20 patients, 4 died, 1 underwent maintenance hemodialysis, 2 had unknown prognosis, 1 did not respond, and 12 experienced partial or complete response.Conclusion Under the premise of surgical treatment and on the basis of adequate anticoagulation and other measures to prevent complications such as thromboembolism, RCC combined with PLA2R-related MN can be observed in an appropriate observation period. If there are no contraindications and the kidney disease is not relieved, hormones, immunosuppressants and biological agents can be given to improve the quality of life and prognosis.