Pelvic lipomatosis is a rare benign entity characterised by excessive deposition of adipose tissue in the perirectal and perivesical spaces. We describe a 43-year-old man with bilateral distal ureteral obstruction secondary to pelvic lipomatosis with concomitant proliferative cystitis resulting in severe hydronephrosis. We performed urinary diversion with percutaneous nephrostomy tube insertion to prevent further renal impairment. The patient was advised close follow-up to monitor the increased risk of bladder adenocarcinoma transformation observed in patients with cystitis glandularis. This report, compliant with the CARE (CAse REport) guidelines for clinical case reporting, presents another case of pelvic lipomatosis associated with acute kidney failure, bilateral hydronephrosis and proliferative cystitis without urodynamic evidence of bladder outlet obstruction that may serve to guide urologists in managing patients with a similar profile.