We report a case of 25-year-old man presenting with a 2-year history of decreased hearing and tinnitus on the right side. Neurological examination revealed profound right retrocochlear sensorineural hearing abnormality, mild right facial spasm and positive Romberg sign. Magnetic resonance imaging (MRI) revealed a giant lesion, hypointense on T1-weighted image and hyperintense on T2-weighted image at the right cerebellopontine angle (CPA). The patient underwent a right suboccipital retrosigmoid craniotomy. Histologically, the cyst depicted characteristics of RCC and was lined by columnar epithelium or stratified squamous epithelium without keratinization. There was gradual improvement of his hearing and neurological deficits 5 months after surgery. There was no recurrence at 60 months follow-up. This case underscores the importance of considering RCC in the differential diagnosis of CPA lesions. The selection of operative approach, based on the relation of cyst wall to normal brain tissue is optimal strategy.