The patient, a 55-year-old man, had undergone surgery for retroperitoneal paraganglioma at the age of 45. In February 2006, he visited our hospital with the chief complaint of metastatic tumors detected by a thorough checkup. Computed tomographic (CT) scan revealed a large tumor in the right kidney hilar region and a left supraclavicular lymphadenopathy. Histopathological and immunohistochemical findings of the biopsy specimen taken from the left supraclavicular lymph node led to the diagnosis of recurrent malignant paraganglioma. 123I-MIBG scintigram showed no radioisotope accumulation consistent with the tumor. From April 2006 to September 2006, he received 8 cycles of CVD therapy (cyclophosphamide, vincristine, and dacarbazine). The tumor temporarily responded and was reduced to one-third in size, but soon it became resistant to CVD therapy. In March 2007, because the tumor had begun to grow, he received the 9th course of CVD therapy, but the tumor response was PD. Subsequently, palliative radiation therapy of 50 Gy in 25 fractions was administered and was temporarily effective. The CVD therapy and radiation therapy were considered to be effective for this case. In May 2008, he died 25 months after the start of CVD therapy.