Objective: Hypoglossal nerve schwannoma, especially when inside the extracranial region, is extremely rare. This report describes a new endoscopic transoral approach for the removal of extracranial hypoglossal schwannoma. Patients and Methods: Three patients (1 male and 2 females, aged 58-63 years) with extracranial hypoglossal schwannoma in the paroccipital condyle and the jugular foramen were treated solely by an endoscopic transoral approach. All patients presented signs of hypoglossal nerve paresis with hemiatrophy of the tongue. In addition, one of them had glossopharyngeal nerve paresis, and vagus nerve paresis was found in another patient. Results: In all patients, tumors underwent successful radical intracapsular removal by the endoscopic transoral approach, as confirmed by the postoperative examinations and MRI. No complications occurred during or after surgery, except that 1 patient had swallowing difficulties and a temporary right vagus palsy during the first day after surgery. All patients were followed up for 6 months, and the lack of any recurrence has been confirmed. Muscle bulk, motor and swallowing functions, and the vagus palsy improved in all patients. Conclusion: The endoscopic transoral approach is a safe and useful surgical technique for the removal of intracapsular tumors, including extracranial hypoglossal schwannomas, which involves minimal invasion. Copyright (C) 2011 S. Karger AG, Basel