Esthesioneuroblastoma is a rare malignant tumor of the nasal cavity that originates from the olfactory epithelium. In this paper a very rare clinical presentation of this tumor is described. The tumor originated from the maxillary sinus and alveolar process, and was independent of the olfactory region. The patient was a 14-year-old girl presenting with facial swelling and nasal obstruction. Paranasal computed tomography showed a mass filling the right nasal cavity, infiltrating the alveolar process, eroding the anterior wall of the maxilla and invading the subcutaneous tissues of the cheek. Fine-needle aspiration and incisional biopsies identified an esthesioneuroblastoma. After neoadjuvant chemotherapy, we performed a right subtotal and left inferior maxillectomy and reconstructed the maxillary defect with a permanent obturator. At 2 years' follow-up the patient is free of recurrence.