We report a new case of chemodectoma of the cauda equina, in a 52-year-old male who presented with low back pain and sciatica, then rapidly developed cauda equina syndrome. Magnetic resonance imaging demonstrated a tumor at the L2-L3 level. Complete excision was performed and the tumor was found to be a chemodectoma. Chemodectomas are rare neural crest tumors that are usually located at the neck. About 70 cases involving the cauda equina have been reported. Most are benign, although local recurrences occur in 4% of cases. A case with a cerebellar metastasis has been reported. Long-term follow-up should be provided.