Primary Ewing sarcoma in the spine is very rare, and the treatment for it is multidisciplinary. There is no consensus regarding the optimal method of local control; however, en bloc resection is associated with an improvement in survival rates. The authors report a case of a 5-year-old girl who initially presented low back pain, and was diagnosed with Ewing sarcoma after being submitted to imaging studies by radiography, magnetic resonance and bone biopsy. A spondylectomy was performed in accordance with the Euro Ewing protocol. At the three-year follow-up, the patient had no restrictions regarding her daily activities, and there has been no evidence of recurrence to date.