Ewing Sarcoma of the head and neck region is an extremely rare entity. Treatment usually involves surgery, chemotherapy and radiotherapy in varying sequences. We present the third case to date of Ewing sarcoma of the larynx in a paediatric population. A 5-year-old boy presented to emergency room with acute respiratory distress. Computerized tomography scan showed a mass in the supraglottis; he was intubated using videolaryngoscope and tracheostomy was avoided, mass was removed by cold dissection. Final histopathologic examination revealed Ewing sarcoma. Further workup showed no systemic metastasis. Patient was advised adjuvant therapy which the family refused. Currently he is doing fine on 2 years of follow-up. © Copyright 2020 by Official Journal of the Turkish Society of Otorhinolaryngology and Head and Neck Surgery.