Neurofibromatosis type II is an autosomal dominant, highly penetrant disease whose hallmark is bilateral vestibular schwannoma. Hearing loss is the most symptom in Neurofibromatosis type II. The patient can also present with tinnitus, disequilibrium, and headache. Cranial nerve symptoms, such as facial numbness or weakness, dysphagia, or hoarseness, can also be present. The authors experienced a case of neurofibromatosis type II having huge retropharyngeal mass, retropharyngeal abscess, and bilateral acoustic neuromas. The infection was controled with aggressive antibiotics with drainage. The huge neurilemmoma in retropharyngeal space was removed successfully via transoral approach. The authors report the case with literature review.