Cholesteatomas (central nervous system epidermoids) can be found intradurally or extradurally in the central nervous system. Extradural intraosseous lesions are most commonly found in the petrous bone. The authors describe a unique case of a clival cholesteatoma in a 64-year-old woman who presented with headaches. No other neurological complaints or physical examination findings were noted. Magnetic resonance imaging showed an expansile lesion centered in the middle portion of the clivus. A large portion of the clivus was eroded. The lesion was explored via a transnasal trans-sphenoidal approach and granular debris was evacuated. The cystic lining was stripped from the surrounding bone, and the bone opening was widely fenestrated. Pathological examination showed keratinous debris with macrophages and an outer lining of benign epithelial tissue consistent with a cholesteatoma (epidermoid cyst). When surgically accessible, these lesions should be excised to prevent a recurrence. If inaccessible, marsupialization may be considered.
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