This case report focuses on a rare glial tumors with an almost exclusive localization in the distal spinal cord or in the sacrococcygeal tissue and which spread metastases only in 17% of cases - myxopapillary ependymoma. We present an unusual case pleuropulmonary dissemination of extraspinal myxopapillary ependymoma in a 28 year old female. It is described the process of differential diagnosis and definitive diagnosis on the basis of the immunohistochemical profile of the cells of the primary tumour and metastases. The diagnosis is finally determined on the basis of strong dispersive expression of GFAP (glial fibrillary acid protein), which is considered diagnostic for this type of tumor. We discuss the likely origin of the tumour as a suspected local recurrence of a sacrococcygeal tumour which was histologically probably misidentified as siringoadenoma in childhood. The discussion summarizes the available information on this low abundance neoplastic disease from the first mention of it in literature on a diagnosed tumour of cauda equina 1956 to the latest findings and the new Classification of Tumours of the Central Nervous System (2007 - WHO Classification of Tumours of the Central Nervous System) with subsequent application to the case of the patient.