Ewing's sarcoma is a primary malignant bone tumor affecting individuals in the second decade of life. Primary sarcomas of the spine are rare and the occurrence of primary Ewing's sarcoma in the spine is very rare. There are 2 types of Ewing's sarcoma of the spine, Ewing's sarcoma of the sacral spine which is very aggressive with a poor prognosis and Ewing's sarcoma of the non-sacral spine which is an extremely rare occurrence. The patient may have a neurological deficit when the tumor extends into the spinal canal, causing compression of the spinal cord. Magnetic resonance imaging (MRI) is very sensitive in diagnosing the tumor and Evaluation of the extent of the tumor. Here, we report the case of a 12-year-old boy who presented with low back pain, weakness of both lower limbs and bilateral spastic paraplegia progressively evolving since 1 month. The CT and MRI scans showed the presence of a tissue lesion process centered on the vertebral body of L1, heterogeneously enhanced after injection of Gadolinium respecting the posterior arch, without bulging of the posterior wall with epiduritis, endocanal extension and spinal cord compression. The patient underwent decompression with surgical biopsy and posterior stabilization of the spine. Histopathology and immunohistochemistry studies confirmed the diagnosis of Ewing's sarcoma and the patient was referred to an oncopediatric center for combined chemotherapy and radiotherapy, but died at home a few days later before the start of treatment.