Trigeminal schwannomas with exclusive extracranial involvement are rare neoplasms. We report a case of a 60-year-old woman who had a slowly growing, large mass on the left cheek. Magnetic resonance imaging examination revealed one 50×33×53 mm mass lesion at left retromaxillary space with involvement of left pterygopalatine fossa, superior orbital fissure, foramen rotundum, vidian canal and left sphenoid sinus; neurogenic tumor from CNV2 was suspected. The tumor was treated by a combined frontotemporal approach and transmaxillary approach (LeFort I osteostomy) under general anesthesia. Diagnosis has been confirmed as schwannoma by post-operative histopathological examination. The patient is scheduled for regular follow-up in our department. This article reports a case of schwannoma and review the literature about the nature and surgical approach of the tumor in this area.