Neurofibroma is a common benign tumor appearing in subcutaneous tissue along peripheral nerves and at neuromedullary junctions. They are, grossly, not distinguishable from neurinomas (schwannomas) or neuromas. These benign tumors frequently occur along peripheral nerves, cerebellopontine angle, brain stem and spinal cord. Acoustic neurinomas from schwann cell within auditory cannal is fairly frequently seen in the field of otorhinolaryngology. There are several reports of neurofibroma originating from tongue, pharynx or larynx. However, neurofibroma from paranasal sinuses are fairly rare and the one developed primarily from ethmoid sinus is quite infrequent. Authors discuss the literatures of neurofibroma with the case report of primary neurofibroma in ethmoid sinus observed in 27 years old male.