BACKGROUND: The primitive neuroectodermal tumors (PNET) of the spine are rare and usually are intramedullary and being reported in children. We herein report an epidural PNET of lumbosacral area presenting with cauda equine syndrome in an adult. CASE REPORT: A 38-year-old woman presented to our emergency room with acute onset lower extremity weakness and urinary incontinency. Emergency MRI revealed an isoitense on T1-weighted and heterogeneously hyperintense in T2-weighted images in epidural lumbosacral area. The patient underwent emergency laminectomy of L-L3 and total resection of the lesion. The patient neurological examination improved dramatically after the surgery and after 6-month of follow-up, she is neurologically intact. The histopathology and immunohistochemistry evaluation revealed PNET. DISCUSSION: Epidural PNET of the spinal column, although are rare, can present with acute neurological deficit. Surgery remains the treatment of choice and immunohistochemistry is required for confirming the diagnosis.