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Bilateral internal auditory canal (IAC) tumors are usually pathognomonic for vestibularschwannomas in the setting of neurofibromatosis type 2 (NF2). Descriptions of bilateral nonschwannomatousIAC tumors, such as metastasis that may mimic NF2, are limited to case reports. Here, we report a case of unusual metastatic bilateral IAC tumor originating from theovary with a rapidly progressive pattern, which we initially suspected to be NF2.