Schwannomas of the descending loopof the hypoglossal nerve (DLHN) have been extremely rare in the literature. We report a young male patient with an approximately 45 mm × 35 mm × 25 mm well-delineated lesion in the carotid triangle. Imaging examination revealed that the mass located anteromedially to the carotid sheath without carotid splaying. Total resection of the neoplasm together with the DLHN was performed, and the pathological diagnosis was a schwannoma. We conclude that the differential diagnosis of tumors in the carotid triangle should consider the possibility of a DLHN schwannoma.