Cavernous angiomas originating in the internal auditory canal are very rare. In the available literature, only 65 cases of cavernomas in this location have been previously reported. We describe the case of a 22-year-old woman surgically treated for a cavernous hemangioma in the left internal auditory canal, mimicking on preoperative magnetic resonance imaging MRI an acoustic neuroma. Neurological symptoms were hypoacusia and dizziness. The cavernous angioma encased the seventh and, partially, the eighth cranial nerve complex. A "nearly total" removal was performed, leaving a thin residual of malformation adherent to the facial nerve. Postoperative period was uneventful; hearing was unchanged, but the patient had a moderate inferior left facial palsy (House-Brackmann grade II) slightly improved during the following weeks. On the basis of the observation of this uncommon case, we propose a revision of the literature and discuss clinical features, differential diagnosis, and treatment.