We describe two cases of metastatic retroperitoneal paraganglioma associated with extradural spinal cord compression. Both occurred in young men; one being metastatic at presentation, the other becoming metastatic 19 years after attempted surgical resection. Despite a long, relatively stable natural history after diagnosis (10 and 19 years, respectively) both had an acceleration of their disease once extradural disease was detected. These cases illustrate the potentially aggressive nature of this disease, the need for long-term follow-up and the effectiveness of a variety of therapies for palliation, and also raise the possibility of "prophylactic" treatment to prevent spinal cord compression.