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A case of a Jehovah's witness affected by an intracanalicular vestibular schwannoma with an extremely fast growth rate is presented. Nine months after presentation, the tumor reached 23 mm in the cerebellopontine angle. A partial removal through a retrosigmoid approach was planned. Because of the presence of a dominant high jugular bulb masquering the internal auditory canal, the intracanalicular portion of the tumor was left in place. The residual tumor grew 12 mm in 2 months. Even after a gross total removal through a middle cranial fossa approach, the tumor recurred, reaching the size of 30 mm in 17 months. A modified transcochlear approach was then performed, and the patient was free of disease at the last radiologic follow-up, 8 months after the surgery. We illustrate our strategy in treating this aggressive benign lesion with unusual behavior.