We report a rare case of pleomotphic adenoma arising from the nasal septum. A 32-year-old woman presented with a 3-week history of left-sided nasal obstruction. Computed tomographic scans revealed an oval mass, measuring about 30 mm in its greatest dimension in the left anterior nasal cavity. The tumor arose from the anterior part of the nasal septum, and was removed endoscopically with endonasal approach. The microscopic finding showed a lobular and duct-like structures consisting of a loose chondromyxoid stroma. This lesion had histological characteristics of a pleomorphic adenoma, and this was confirmed by immunohistochemical expression of cytokeratin and S-100 protein. Her post-operative course was uneventful, and she is currently free from the disease 8 months after surgery. Diagnosis, clinical behavior and treatment of pleomorphic adenoma of the nasal septum are reviewed from perusal of the literature. (C) 2004 Elsevier Ireland Ltd. All rights reserved.