Schwannomas are neurogenic neoplasms rarely found in the sinonasal tract, where localization to the nasal septum is exceedingly rare (only 11 cases have been described in the western literature). We report the case of a 29-year-old white male with a schwannoma completely filling the left nasal fossa and arising from the bony part of the septum. A computer tomography (CT) scan and a biopsy suggestive of benign schwannoma were obtained before the lesion was removed by a degloving approach. The preoperative diagnosis of nasal septum schwannoma was confirmed. The patient is asymptomatic and without endoscopic evidence of recurrence 7 years after surgery. A review of the literature with particular emphasis on the clinical presentation, histological features, differential diagnosis and therapeutic options for such a rare lesion is included.