Schwannomas of the descending loop of the hypoglossal nerve are very rare. Existing literature of the schwannoma of the descending loop of the hypoglossal nerve is limited to two previously reported case. They are slow-growing tumors that may masquerade a carotid body tumor. We herein described a rare case of schwannoma of the descending loop of the hypoglossal nerve in the s right latero-cervical region with diagnostic imaging and histopathological findings. A 37-years-old woman has had a palpable firm, mobile mass in the right latero-cervical region, of imaging, MR images showed homogeneous hypointensity on T1-weighted imaging (T1-WI), heterogeneous hyperintensity on T2-WI, and heterogeneous enhancement on contrast-enhanced T1-WI. Diagnostic imaging using computed tomography (CT) and magnetic resonance imaging (MRI) was suspected of Chemodectoma or neurogenic tumor. At operation, a 4 cm mass arising from the descending loop of the hypoglossal nerve of was resected en bloc with the loop itself; Final diagnosis was confirmed on the basis of histopathological finding and intraoperative findings. Postoperative course was uneventful and the patient is free from disease recurrence at tree-year follow-up. En bloc resection remains the real curative treatment of Schwannomas, ensuring unlimited freedom from disease, although causing functional impairment which may be significant. Nonetheless recurrence should be prevented as, besides requiring reintervention, it may harbor a malignant evolution towards sarcoma. Schwannomas of the descending loop of the hypoglossal nerve may masquerade a chemodectoma of the carotid bifurcation and can be curatively resected without any functional impairment. This case confirmed the differential diagnosis on the basis of the intraoperative finding that the tumor was continuous with the hypoglossal nerve.