Primary spinal primitive neuroectodermal tumors (PNETs) of the extradural space are very rare; only 10 cases have been reported in the English language literature. The histopathological diagnosis of primary spinal PNETs has been discussed for many years. These tumors have a rapidly progressive course, and there is no current consensus on the optimal therapeutic approach for these patients. The authors present a case of primary PNET located in the lumbar extradural space in a 13-year-old girl and report the clinical, radiological, histopathological, and surgical findings. They compare their findings with those from the other 10 cases reported in the literature and review relevant literature.