The prognosis of patients with Ewing sarcoma is poor. Peripheral blood stem cell transplantation (PBSCT) is a kind of hematopoietic stem cell transplantation combined with extremely high-dose chemothrapy. We report two cases of Ewing Sarcoma-one had a huge primary left calf tumor and another a huge primary submaxillary tumor with lung metastasis-successfully treated with extremely high-dose chemotherapy with PBSCT. We suggest that PBSCT combined with chemotherapy is efficacious and could be beneficial in improving the prognosis of highly chemoresponsive tumors such as Ewing sarcoma.