Esthesioneuroblastoma (ENB) is a rare malignant neoplasm originating from the olfactory epithelium located in the upper part of the nasal cavities. The treatment is not yet well codified; external surgery and radiotherapy were the standard treatment. We have reported a case of a 20-year-hold patient treated with neoadjuvant chemotherapy, surgery, and then adjuvant chemotherapy. The prognosis of these malignant tumors is similar to that of ethmoid adenocarcinomas and better than that of squamous carcinomas of the facial bone structure. 5-year overall and disease-free survival averaged 45%.