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An arachnoid cyst arising within the internal auditory canal, or within the cerebellopontine angle and subsequently extending into the internal meatus and enlarging it, is a rare occurrence. Nevertheless, the neurootologist and the neurosurgeon have an interest in its existence because its clinical manifestations are identical with the ones produced by a schwannoma, involving overwhelmingly the cochleovestibular nerve in that region. An equally rare observation in that location is the presence of ectopic neuroglial tissue. The two can occur independently or in combination. Examples of such lesions are presented, and their possible pathogenesis is discussed.