A rare case of malignant paraganglioma of the urinary bladder with metastasis to a lymph node in a 12-year-old girl is reported, and eight other previously recorded cases are reviewed. Headache, fainting, and hypertention initiated by voiding were the most prominent clinical symptoms. Hematuria was Present in three cases and aided cystoscopy were most diagnostic. Cystography, pelvic angiogtaphy, and cystoscopy were most diagnostic. Segmental cystectomy, when feasible. was the preferred surgical treatment. The prognosis was slightly better than for other forms of extraadrenal pheochromocytomas.