We report the case of a 58-year-old man with metastatic tumors 13 months after the initial surgery for paraganglioma at the left adrenal gland. A CT scan revealed a large tumor at the right scapula and abdominal paraaortic lymph nodes, and the patient received combination therapy of cyclophosphamide, vincristine and dacarbazine (CVD) every 3 to 4 weeks. After 11 courses, the serum catecholamine level was reduced to the normal range. The metastatic tumors showed optimal reduction in size, and the patient remains alive with no symptoms of the disease one year after the primary chemotherapy. The combination therapy of cyclophosphamide, vincristine and dacarbazine is considered a feasible and effective chemotherapy for metastatic malignant pheochromocytoma.