Extracranial meningiomas of the paranasal sinus are the rare tumors of unclear etiology. We report a case of primary extracranial meningioma arising from the roof of the sphenoid sinus. A 60-year-old female presented with 5-month history of right visual disturbance. Imaging studies showed a mass in the sphenoid sinus extending into posterior ethmoid sinus. The tumor was resected completely including the affected bone of anterior cranial base via the endonasal endoscopic approach. Pathological diagnosis was meningothelial meningioma. On the basis of imaging features and intraoperative findings, the tumor was diagnosed as primary extracranial meningioma of the sphenoethmoid sinus. Recent advancement of skull base surgery technique allows removing this rare tumor completely and safely without complications such as cerebrospinal fluid leakage. This is the first report of the management of spheno-ethmoid sinus meningioma with the skull base surgery technique.