Ewing sarcoma (ES) is an aggressive, primary bone malignancy with occasional soft tissue extension. Purely extraosseous ES is rare. A primary intraspinal, intradural ES without bone involvement is exceedingly rare. ES may be differentiated from other primitive neuroectodermal tumors by molecular analysis. The authors report the case of a 14-year-old female who suffered an acute neurologic decline from a hemorrhagic, intraspinal, intradural ES. The patient has been tumor free for 2 years after the initial emergency surgery. Our management of the patient and a review of the literature are provided. Considering only those cases with molecular or genetic confirmation of ES, our patient is the fifth pediatric case reported in the English literature.