We report a case of a cerebellopontine angle cholesteatoma whose initial sign was benign paroxysmal positional vertigo (BPPV). Positional vertigo caused by a central pathology is extremely rare and is usually accompanied by other suspicious features. In this case, there were no additional neurotologic symptoms or signs. The only abnormalities were seen on Dix-Hallpike testing, but because they were not consistent with a diagnosis of BPPV, the decision was made to proceed to imaging. Diagnostic rigor is required when evaluating positional vertigo, as with all symptoms of imbalance, if such cases are not to be overlooked.
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