Base of tongue schwannomas are exceedingly rare and therefore often are not immediately included in the differential diagnosis and treatment of oropharyngeal tumors. After a thorough review of the English literature, we found only 6 previously reported cases of tongue base schwannomas. We are contributing a report of a 37-year-old woman with progressive dysphagia, dysarthria, and large tongue base schwannoma. Diagnosis was confirmed by imaging studies and biopsy followed by surgical excision designed to preserve nerve function. A number of surgical approaches have been described for tongue base schwannomas. Each has its own degree of postoperative morbidity. The use of a unilateral transcervical incision with blunt dissection was simple and quickly accomplished with protection of nearby nerves. Histologic identification of Antoni A and B areas along with strong and diffuse staining with S-100 stain pathologically completed the diagnosis of schwannoma.