This report describes an extremely rare case of malignant extra-adrenal pheochromocytoma of urinary bladder with multiple bone metastases in a hormonally inactive 38-year-old man, who initially presented as right shoulder pain, frequency, dysuria and microhematuria. A combined-modality treatment plan of partial cystectomy with post-operative palliative radiotherapy for bone pain and subsequent systemic chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) was performed. Although the patient survived the surgical intervention and the cytotoxic chemotherapy for at least 9 months, the bone metastatic lesions showed unsatisfied response after undergoing 3 courses of CVD chemotherapy.