Abstract
Epithelioid sarcoma is a rare and highly malignant soft tissue neoplasm that most commonly occurs in the long bones. This uncommon tumor has a poor clinical outcome, and the modality of its treatment has not yet been fully established. The authors report an extremely rare presentation of epithelioid sarcoma in the cervical spine, along with its clinical progression, imaging, and pathology. The patient underwent three surgical procedures and adjuvant radiochemical management. He survived for 25 months with a good general condition and adapted well to his social activity. Systemic metastasis was not found, but the patient died of respiratory failure due to direct tracheal invasion of the tumor.