Epidermoid cysts are rare congenital lesions that comprise approximately 1% of all intracranial tumors. The cerebellopontine angle (CPA) is involved in about 40-50% of these cases. Previous meta-analysis study showed that hearing loss, trigeminal neuralgia, vertigo, facial palsy or headache is usually main symptom. A 58-year-old male presented with vertigo and a sudden onset of sensorineural hearing loss (SNHL) in the left ear. The audiograms revealed a severe left-sided SNHL. The patient also complained severe dizziness. Brain MRI showed a 2 cm sized cystic mass with iso-intense signal on the T1 weighted image (WI), a slightly hyper-intense signal on the T2WI and thin rim enhancement after the administration of contrast in the left CPA cistern area. The patient was treated with a 10-days course of prednisolone followed by a course of vestibular rehabilitation therapy. Hearing remained unchanged. Vertigo symptom was substantially recovered. We report here on an unusual case of sudden SNHL due to epidermoid cyst in the CPA lesion with a review of the related literatures.