Esthesioneuroblastoma (ENB) is a rare malignancy, representing only 3% to 6% of all sinonasal malignancies. A wide array of treatment options for ENB have been described in the literature, but prospective clinical trials are absent given the tumor's rarity and natural history. Delay in diagnosis leading to an initial advanced stage of presentation is common secondary to the clinically hidden primary site at the anterior skull base. This article presents data from the current body of literature and reviews the advocated roles for surgery, radiation therapy and chemotherapy.