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Intramedullary epidermoid tumors are rare. To our knowledge, this is the first description of a multiple intraspinal epidermoid tumor, including the intramedullary conus medullaris and an intradural lesion in the lumbar spine. The authors report the case of a 43-year-old woman presenting with progressive paraparesis. A thoraco-lumbar lesion was confirmed as an epidermoid cyst from histopathology. The clinical, radiological and surgical features, as well as a brief critical review of the literature are included. Key words:Multiple epidermoid cysts;Intramedullary tumor.