Paragangliomas are tumors arising in the paraganglia. Involvement of the spine is less common, and usually takes the form of intradural compression of the cauda equina. We report here a case of a 60-year-old man with recurrent and progressive pain of his sacral and perineal area, accompanied by occasional rod and perineal hypoesthesia on admission. He underwent laminectomies of the vertebral bodies S1 and S2, and an en bloc resection of the tumor. Postoperative histopathological examination revealed a paraganglioma. Postoperative staging showed no pathological abnormalities, and no tumor recurrence after one year. Even though rare, the paraganglioma of the sacral spinal canal should be considered in the differential diagnosis of tumors occurring in the spine.