Esthesioneuroblastoma is a rare malignant neoplasm in the olfactory region of the nasal cavity and anterior skull base. Diagnosis and staging require anatomic imaging and careful pathologic assessment. Standard treatment is anterior craniofacial resection with postoperative irradiation. The role for chemotherapy is not defined, but is generally for the most advanced cases and used in the neoadjuvant setting and/or postoperatively with irradiation. Prognosis is favorable; however, metastasis rates remain relatively high. Regional and distant metastasis portends a poor outcome. Intensity-modulated radiation treatment and endoscopic surgery have reduced morbidity, but outcomes with these techniques must be fully evaluated.