We present an association not previously described in which the rare entity of paraganglioma of the cauda equina was found in association with syringomyelia of the cervico-thoracic cord. The patient, a 50-year-old woman presented with a long history of lower back pain with recent onset of sciatic pain and altered sensation in the right leg. Surgical resection of the tumor with intraoperative syringostomy of the syrinx has led to eradication of all symptoms and at 5 months shows no evidence of residual pathology.