A 35 years old male patient presented with serous nasal secretion, relapsing epistaxis and a slowly progredient nasal obstruction. Clinical examination and CT scans showed a tumor occupying the left middle meatus and the whole nasal cavity displosing the nasal septum to the right side. The tumor was resected by a midfacial degloving approach. Histological examination showed a neurinoma. By means of the presented case report and a review of literature, diagnosis and treatment of this rare nasal tumor are discussed.