Esthesioneuroblastoma is a rare nasal tumor arising from olfactory epithelium. The clinical course of this rare tumor is characterized by local destruction with aggressiveness and less commonly distant metastasis. The diagnosis of esthesioneuroblastoma can be made by the finding of plexiform intercellular neurofibril by light microscopy and the finding of secretory granules and neurites by electron microscopy. Recently, we have experienced one case of esthesioneuroblastoma in the nasal cavity extending into the maxillary sinus, ethmoid sinus, and lower part of frontal lobe area. So, we report this case with review of the literature.