Abstract Myoepithelioma is defined as myoepithelial cells composed of tumor with solid, myxoid, and reticular growth pattern by the World Health Organization. It is a rare neoplasm that arises in the major salivary gland or minor salivary gland comprising approximately 1% of all salivary gland tumor. This neoplasm occurs in the parotid gland and soft palate mostly, which can be found in other organs. However, up to now, myoepithelioma of nasal septum has not been reported in English medical literature. Histologically, it is grouped as spindle, epitheliod, reticular, and clear cell types. Spindle cell type is the most common. In this case, a 40-year-old woman presented with complaining of nasal stuffiness and accidentally found right nasal cavity mass. Endoscopic surgery for excision of the mass was performed under local anesthesia, which was confirmed as myoepithelioma. After surgery, the patient's symptom was disappeared and there has not been sign of recurrence for 6 months. We present a rare patient of myoepithelioma of the nasal septum with review of relevant literature.