We report a rare case of arteriovenous malformation(AVM)of the parotid gland associated with Cowden disease successfully treated with preoperative embolization followed by surgical removal. A 39-year-old man with a history of Cowden disease presented with a pulsating and growing mass on his left lower jaw. Contrast-enhanced computed tomography(CT)and angiography revealed a high-flow AVM in the deep lobe of the left parotid gland. After intravascular embolization of the feeding arteries, surgery was performed using the NIM-response®3.0, facial nerve monitoring system. The AVM was almost completely removed and the facial nerves were morphologically preserved. Interestingly, the intraoperative findings revealed that the enlarged vasa nervorum of the facial nerve also fed the AVM. Although left facial nerve palsy appeared after the surgery, the nerve function gradually improved over one year. No recurrence of the AVM has been observed for one year.