Hypoglossal schwannoma is a rare tumor this frequency approximately less than 5% all non-vestibular schwannomas. Also, it may be sign of neurofibromatosis type 2. Usually, the tong deviation in side of the tumor is the first symptom of the disease. When the tumor size is increased, bulbar disorders and cervico-occipital pain are develop. MRI is the main method of diagnostic. Until the 1970s, mortality after surgical removal reached 50% and was due to bulbar and respiratory dysfunctions. Nowadays, the cause of death remains the same. After introduction stereotactic radiotherapy into clinical practice total removal is not necessary. The purpose of surgery stay is removal of intracranial part of the tumor and decompression of the brainstem (subtotal removal). Radiotherapy or radiosurgery is performed on the intracanal fragment of the tumor. The case of successful surgical treatment of a patient with hypoglossal schwannoma is presented in this article. Subtotal removal was performed via median suboccipital approach. Radiosurgery is planned for residual part of the tumor, located in the hypoglossal nerve channel.