BACKGROUND: Approximately 95% of tumors occurring within the internal auditory canal (IAC) are vestibular schwannomas. Many undergo stereotactic radiation without definitive tissue diagnosis. Rare IAC tumors are not all radiosensitive and are poorly described. METHODS: Between 1992 and 2015, 289 consecutive patients with IAC lesions operated on were reviewed retrospectively. RESULTS: Fifteen patients (5.2%) (16 operations) had unusual histologic findings, including nonvestibular schwannomas (2 facial schwannomas, 2 cochlear schwannomas, 2 intermedius schwannomas), 3 meningiomas, 3 cavernous hemangiomas, a mucosa-associated lymphoid tissue lymphoma, an arachnoid cyst, and a lipochoristoma. None of these rare tumors could be identified before surgery. Three operative approaches were used: the retrosigmoid approach, middle fossa subtemporal approach, or translabyrinthine approach. Few complications occurred, including facial nerve palsy, loss of hearing, and vestibular function. Five-year average follow-up revealed one patient with recurrence. CONCLUSIONS: Clinical examination and imaging alone were insufficient to correctly identify these tumors. Definitive pathologicdiagnosis should be strongly considered to help tailor treatment.