Olfactory esthesioneuroepithelioma is a rare malignant tumor in adults, and first case was reported by Berger et al. in 1924. Since the first description of this tumor there have been 104 subsequent cases reported in the literature (Skolnik, 1966). Interest in this tumor is stimulated by its rarity and by its unique origin from the sensory cells of olfactory mucosa. Histologically, its important to differentiate esthesioneuroepithelioma from other tumor of the nasal cavity because of its striking radiosensitivity. This seems to be the youngest case not found in the literature yet. The patient was a 6-year-old female who had nasal stuffiness of the left side, mucopururent discharge, headache and exophthalmus of 1 month's duration. Visual acuity was not changed. At operation, this tumor was located all over the left nasal cavity and ethmoid sinus. It was difficult to extirpate the tumor mass due to friable, soft and polypoid nature. A brief review of the literature was made.