Olfactory neuroblastoma (ONB) is originated from the olfactory neuroepithelial cells of the nasal cavity and it accounts for <3% of tumors originating in the nasal cavity and paranasal sinus. Intracranial extension and orbital involvement have been shown to be independent risk factors associated with poorer outcomes. Treatment options are surgical excision or surgery combined with a radiotherapy (RT) and/or chemotherapy. The present study reports the case of a 57-year-old patient with a mass in the left nasal cavity since 6 months that was presented as progressive nasal obstruction and intermittent left-sided epistaxis associated with anosmia and loss of vision. A biopsy and immunohistochemistry of this mass was confirmed high grade olfactory neuroblastoma. A decision was made to start neoadjuvant chemotherapy with cisplatin and etoposide and had good response. This case report illustrate that neoadjuvant chemotherapy is effective treatment for high grade olfactory neuroblastoma. The literature for ONB and the treatment of the tumor are also discussed.