Schwannoma is a tumor derived from Schwann cells that most commonly affects the vestibular nerve. Hypoglossal nerve schwannomas account for a small percentage of non-vestibular head and neck schwannomas and are differentiated from vestibular schwannomas by their location and symptomatology. Important differentials include paragangliomas, metastatic lymph nodes, and carotid artery aneurysms. Herein, we present the case of an elderly female patient presenting with a well-defined, mobile, and non-tender 3.5 cm soft mass at level II of the right neck. A computerized tomography scan revealed a poorly enhancing and heterogeneous tumor. As the patient had symptoms of hoarseness, we initially diagnosed a vagus nerve tumor, which was later confirmed to be a schwannoma of the hypoglossal nerve. The patient underwent surgical management and developed postoperative ipsilateral tongue weakness. The phonation and tongue weakness were gradually compensated under follow up. The case report illustrates a rare case of a neck mass diagnosed as a hypoglossal schwannoma with its treatment and prognosis. We also reviewed the recent literature for discussion. (J Taiwan Otolaryngol Head Neck Surg 2020; 55:218-224)
