Mucoceles are relatively common cystic lesions of the paranasal sinuses. However, mucocele of the nasal septum is extremely rare. We report a case of a mucocele present in this unusual location. Mucocele of the nasal septum should be considered in the differential diagnosis of a mass of the nasal septum and/or median canthal region. Nasal septal mucocele can be effectively treated with endoscopic marsupialization or complete excision.