Esthesioneuroblastoma is a rare neoplasm of the nervous system that appears in the paranasal cavities derived from the olfactory neuroepithelium, located in the cribriform region of the nasal fossa, and can invade the sinuses of the face, skull base and orbital region. Kadish classification is the most widely used to stage it according to its extension. The symptomatology is unspecific, and it may present progressive unilateral nasal obstruction, epistaxis, anosmia, headache, sinusitis, diplopia, and orbital proptosis. The diagnosis is based on the lesion biopsy, immunohistochemistry, and ultrastructural findings. Excision of the tumor is described as the treatment of choice that may be associated with radiotherapy and chemotherapy. This is a case report of an olfactory esthesioneuroblastoma in a patient admitted to the hospital complaining of orbital proptosis who, after a computed tomography of the skull, showed a suspicious lesion. After the biopsy and the anatomopathological study, a protocol with neoadjuvant chemotherapy was initiated, with the subsequent excision of the tumor. Despite the lack of other symptoms, an advanced stage of the neoplasm was identified, which reflects the importance of investigating less common complaints and thus facilitating the diagnosis and early treatment.