The nasal septum is one of the rarest sites of origin of schwannomas. Recently, as the authors experienced a patient with nasal septal schwannoma completely removed by transnasal endoscopic approach, the authors tried to review all the literatures reported since Berlucchi's report in 2000. To the best of our knowledge, only 18 patients with nasal septal schwannoma in the literatures written by English-language have been reported. The purpose of this study is to describe our recent experience and thoroughly review our collected patients with nasal septal schwannoma with particular emphasis on therapeutic options. Additionally, it is to describe the changes in clinical symptoms, diagnosis, and treatment of nasal septal schwannoma by comparing our data with Berlucchi's data. Based on our review, the authors suggest some conclusions. First, since common symptoms were nonspecific like other intranasal tumors, the authors should keep in mind that nasal septal schwannoma is included in the differential diagnosis of unilateral nasal mass pathologies with a high degree of suspicion on the basis of the endoscopic findings. Second, although there is no diagnostic computed tomography (CT) finding of nasal septal schwannoma, CT scan with contrast enhancement is helpful in defining the origin and location of the tumor and the involvement of vital structures. Third, combined immunohistochemical staining for S-100 protein, calretinin, and CD56 in addition to the light microscopic findings can be very useful as a simple, easy, and reliable method for definite diagnosis. Last, transnasal endoscopic surgery is the most suitable approach for treating any size and location of nasal septal schwannomas.