Cases of primary spinal epidural Extra-osseous Ewing's sarcoma (EES) are rarely seen and a good prognosis for EES cannot be expected since a high incidence of local recurrence and metastasis frequently occur. We present a case of cervical spinal epidural EES in a 7-year-old girl with long survival after tumour resection. She also received adjuvant treatment with peripheral blood stem cell transplantation (PBSCT). This management successfully improved her condition and she was in complete remission without neurological deficit 60 months after surgery. Such a good prognosis of EES in the cervical spine is very rare. It is likely that magnetic resonance imaging (MRI) lead to a diagnosis of this rare type of EES at an early stage of the disease. PBSCT could be useful as an adjuvant to prolong the period of complete remission.