Neurilemmoma is a benign, well-encapsulated neurogenic tumor that arises in cranial, peripheral, or autonomic nerves that have nerve sheaths containing schwann cells. From 16 to 45% of all neurilemmoma cases occur in the head and neck region and only about 4% of those cases are found in the nose and paranasal sinuses, but very rarely found in the nasal septum. Recently, we experienced a case of isolated neurilemmoma which arose from the nasal septum of a 30-year-old female patient who complained of nasal obstruction and postnasal drip. The mass was broadly based on the right anterior portion of the nasal septum and confined to the nasal cavity, displacing the lateral wall of the nasal cavity and middle turbinate, laterally and posteriorly, respectively. The tumor mass was completely excised through transnasal endoscopic surgery under general anesthesia. The final pathological diagnosis viewed under a microscope after H&E and anti S-100 protein stain was a benign neurilemmoma in which Antoni A type and B type both existed. This report presents a case of neurilemmoma which arose from the nasal septum with reviews of the clinicopathologic features.