Myxopapillary ependymomas are low grade tumours that are known to recur locally even after complete excision, but spontaneously widespread dissemination is extremely uncommon. We report the unusual case of a 14-year-old boy suffering from progressive back pain. Magnetic resonance imaging showed spinal lesions associated with intracranial widespread leptomeningeal dissemination. The patient underwent surgical removal of the tumour. Microscopic examination showed histological and immunohistochemical features consistent with a diagnosis of myxopapillary ependymoma. We have performed a careful review of the literature on spinal myxopapillary ependymoma and metastasis. To the best of our knowledge, this present case seems to be the first report on spontaneously widespread leptomeningeal dissemination of a spinal myxopapillary ependymomas without previous surgical intervention.