We report an extremely rare case of mucoepidermoid carcinoma of the nasal septum. A patient with a history of right-sided nasal obstruction presented to our clinic. Clinical examination revealed a mass in the right nasal cavity originating from the nasal septum. Biopsy revealed a high-grade mucoepidermoid carcinoma. The patient was treated with surgical resection only. Surgery alone might be suitable for small tumors when direct inspection of the surgical site is feasible to detect early recurrence.