Myxopapillary ependymoma occurs almost exclusively in the region of the cauda equina. Myxopapillary ependymoma is a benign tumour, with a tendency for slow growth and local recurrence. Intracranial and systemic metastases have been reported. Treatment usually includes maximal resection. Adjuvant radiotherapy is indicated in the case of incomplete resection. Although cases of prolonged disease-free survival after radical surgical resection have been reported, local recurrence is often observed after a few or even many years. Patients with myxopapillary ependymomas should be followed clinically and radiologically for a long time because of the incidence of late recurrence and dissemination. It is still not decided how long and how often follow-up control should be carried out. We report the case of a patient with extraneural metastases of the cauda equina myxopapillary ependymoma observed for a period of twenty years.