We describe a patient with dural arteriovenous fistula (DAVF) treated with Onyx-18 who developed isolated hypoglossal nerve palsy. This is the first case of isolated hypoglossal nerve palsy caused by Onyx-18 embolization. This complication suggests that over embolization with Onyx-18 in the treatment of hypoglossal canal DAVFs should be avoided, and transvenous embolization may be safer. Furthermore, prednisolone therapy should be carried out in the prophase of nerve palsy.