We report a rare case of pleomorphic adenoma arising from the nasal septum. A 37-year-old woman presented with a 1-year-history of right-sided occasional epistaxis. Computed tomographic scans revealed an oval mass in the right nasal cavity. The tumor was removed endoscopically with endonasal approach. The microscopic finding showed numbers of myoepithelial cells and duct-like structures consisting of loose myxoid stroma. This lesion had histological characteristics of a pleomorphic adenoma, and this was confirmed by immunohistochemical expression of cytokeratin, S-100 protein and SMA. Her post-operative course was uneventful, and she is currently free from the disease 1.5 years after surgery.