Hypoglossal nerve (HyN) schwannomas are uncommon tumors. The leading symptoms are unilateral tongue weakness and atrophy. The neoplasms are diagnosed by MRI imaging. The surgical treatment of hypoglossal schwannomas (HS) is challenging because the tumor is closely related to brainstem structures, cranial nerves, and arteries of the vertebrobasilar system. Their removal is associated with a high risk of patient disability. This report presents a clinical case study of a 68-year-old patient with an unusual clinical presentation of intradural HS. There were no signs of tongue weakness or atrophy observed in the patient. After the tumor was diagnosed, it was radically removed. This case highlights the importance of early detection and complete microsurgical resection of schwannoma to prevent neurological deficits and ensure a favorable postoperative outcome, thus maintaining the patient's quality of life. Keywords: Hypoglossal nerve, schwannoma, tongue weakness and atrophy, microsurgery.