Abstract: Chordomas mainly occur in the axial bones, the most common in the base of the skull and the sacrococcygeal region, and are rare in other parts. The author reported a case of chordoma in the right nasal cavity and ethmoid sinus confirmed by histopathological results. The clinical symptoms, preoperative CT and MRI imaging findings, surgical findings and postoperative histopathology immunohistochemical examination results were analyzed. The characteristics of the disease and the differential diagnosis through literature review have deepened the understanding of the disease, provide valuable reference for clinical diagnosis and treatment.