We present an extremely rare case of epithelial-myoepithelial carcinoma (EMC) arising in the nasal cavity. The patient was a 56-year-old Japanese male with a polypoid tumour arising from the nasal septum. Histopathological examination revealed the tumour to consist of a solid proliferation of clear-cells and, in some areas, small or elongated duct structures with a double-layered arrangement of inner cuboidal cells and outer clear-cells. Dual differentiation toward myoepithelial and ductal cells were confirmed immunohistochemically. The occurrence of EMC in the nasal cavity is possible and this entity should be generally recognized by surgical pathologists, not only those engaged in head and neck surgery.