Schwannomas are benign peripheral nerve sheath tumors. These are rare in the oral cavity (1%). The most common site of involvement in oral cavity is the tongue. Posterior third of tongue is not frequently involved. The aim of this paper is to present a case report of base tongue schwannoma and review literature of this rare tumor. Data from literature were analyzed for age, gender, presenting symptom, size at presentation, and surgical approach. We report a case of 26 year-old male who presented with swelling posterior 1/3rd tongue and change in quality of voice. He was evaluated for the same with MR and incision biopsy and was planned for surgery. Surgery was abandoned at a district hospital due to difficulty in intubation. At our center he underwent fibro optic bronchoscopy guided intubation followed by general anesthesia. He underwent excision of mass using left paramedian lip spitting approach with mandibulotomy and mandibular swing. Tumor was excised in toto. His postoperative recovery was uneventful. Literature review between 2001 and 2012 was done. 15 cases of base tongue schwannoma were identified. The most common age group involved was between 30-40 years. There was a slightly higher incidence in females. All patients were symptomatic at presentation. Most common complaints were related to swallowing and throat pain. Most patients underwent transoral excision of the tumor.