A 6-year-old girl was found to show a presacral epidermoid cyst which had a communication with the spinal canal. The patient was admitted as a case of meningitis, but squamous cells and cholesterin crystals were observed in the cerebrospinal fluid (CSF), and the CSF cholesterol level was very high. Diagnosis was established by myelography, and a cyst measuring 4.5 × 6.0 × 4.0 cm wide was totally excised surgically. A comparative discussion of this case and other reported cases of dermoid and epidermoid cysts in the central nervous system is given.

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