Background: Pelvic lipomatosis (PL) is a rare condition that is characterized by excessive growth of fat in the pelvic cavity. Studies have yet to systematically review surgical treatments for PL. Objectives: To provide a reference for selecting reasonable surgical treatments for PL patients according to previous literature on the surgical treatment of PL. Design and methods: We conducted this systematic review in accordance with the Preferred Reporting Items for a Systematic Review and Meta-Analysis (PRISMA) of Individual Participant Data guidelines. Literature on PL published from 1968 to 2022 was retrieved from the PubMed and EMBASE databases. Data were collected and analyzed independently by two independent investigators. Results: A total of 42 studies, involving 231 patients with PL, were included in the analysis. The surgical treatments included transurethral resection (TUR) (48.5%), ureteral stent placement (11.7%), percutaneous nephrostomy (1.3%), ureterocutaneostomy (1.3%), ureteral reimplantation (10.4%), ileal conduit (13%), and allograft kidney transplantation (0.4%). After excluding patients with unclear prognoses, 42.9% of patients showed improvement in lower urinary tract symptoms (LUTS) after TUR. Ureteral stent placement provided relief of hydronephrosis in 62.5% of PL patients. Percutaneous nephrostomy resulted in stable renal function in 33.3% of PL patients, while ureterocutaneostomy led to remission of postoperative hydronephrosis in 33.3% of PL patients. After ureteral reimplantation, 70.8% of patients experienced relief of hydronephrosis or had stable renal function. Ileal conduit led to remission of hydronephrosis, alleviation of symptoms, or maintenance of stable renal function in 83.3% of PL patients. One patient with PL had stable renal function after allograft renal transplantation. Conclusion: The surgical treatments for PL include TUR, ureteral stent placement, urinary diversion, and allograft renal transplantation. However, the choice of surgical method should be determined after comprehensive consideration of the patient’s condition.