Paraganglioma is a rare neuroendocrine tumor that arises from the autonomic nervous system. The urinary bladder paraganglioma accounts for less than 0.1% of bladder tumors. It remains a challenging entity to diagnose and treat due to its rareness and the lack of specific symptoms. Treatment modalities include transurethral resection and cystectomy (partial or total). The authors report a new case of an isolated paraganglioma of the urinary bladder in a 52-year-old female patient that underwent partial cystectomy. This case aims to remind the clinical, histological and therapeutic features of this rare tumor.