Chordomas are malignant, nonepithelial neoplasms derived from notochordal tissue. A primary chordoma of the nasal cavities and paranasal sinuses is extremely rare compared with clival chordomas, which often present as nasal masses after spreading anteriorly. Only a few cases of primary chordoma of the nasal cavities and paranasal sinuses have been reported in the literature. We report a case of a primary chordoma of the lateral nasal wall. Pathologic diagnosis was obtained using an intranasal endoscopic excision. As in our patient, a primary chordoma of the nasal cavity or paranasal sinuses may present with symptoms related to mechanical obstruction secondary to the tumor mass. We summarize our case in the context of the other reported cases, and we discuss treatment options, natural history, and prognosis.

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