Esthesioneuroblastoma is a rare and malignant upper nasal cavity neoplasm involving the anterior skull base. Treatment includes surgery, radiotherapy, chemotherapy, or a combination. The ideal treatment modality has yet to be determined. Esthesioneuroblastoma often lies in proximity to the optic nerves, optic chiasm, and the orbit. Resection risks damaging these critical structures, and radiotherapeutic techniques, similar to those applied for paranasal sinus tumors, may damage these vital structures and result in late sequelae such as blindness and cortical necrosis. Management strategies for this neoplasm lack uniformity, and there is no universally accepted staging system. In this paper the authors discuss the clinical presentation, radiological and pathological features, and treatment of this rare, malignant skull base neoplasm, as well as review the literature. They also present their results and treatment regimen, which includes preoperative radio- and chemotherapy or 1) craniofacial resection if the lesion has a significant intracerebral component, or 2) frontal sinus resection if little intracranial extension exists.