Schwannomas are benign tumors arising from the nerve sheaths of peripheral, autonomic and cranial nerves. Between 25% and 45% of all schwannomas occur in the head and neck region but only 4% involve the nasal cavity and paranasal sinus. Moreover, schwannoma of the pterygopalatine fossa is extremely rare. We present a case of schwannoma of the pterygopalatine fossa in a 43-year-old woman. The patient was surgically managed with endoscopic transnasal approach combined with transantral approach. After 16 months, the patient shows no evidence of recurrence.