Neurilemmoma originating in the ethmoid sinus is rare. We had a patient with neurilemmoma arising from the left anterior ethmoid sinus. A 60-year-old woman, with a painless swelling in the nasal root for the last 2 years, was examined. A CT scan image showed a solitary mass in the bilateral anterior ethmoid sinus and frontal sinus. Under general anesthesia, the tumor was removed completely with bilateral lateral rhinotomy adding transverse incision. Histological examination revealed a typical picture of neurilemmoma demonstrating Antoni A type. This case showed good postoperative course.