We present a very unusual case of an acoustic neuroma involving the left cochlea and internal auditory canal of a 24-year-old man. Clinical suspicion was aroused when the patient presented with a left total sensorineural hearing loss and continuing vertigo. The diagnosis was made pre-operatively with MRI after initial CT scanning was normal. The tumour was removed via a transotic approach. This case report demonstrates the MRI features of an intracochlear schwannoma and emphasizes the importance of MRI in patients with significant auditory and clinical abnormalities with normal CT scans of the relevant region.