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The epidermoid cyst is a benign congenital tumor and originating from the ectoderm. It occurs in the third and fifth decade of life, corresponding approximately to 0.2 to 1.8 % of intracranial tumors. The type of intradiploic epidermoid cyst is rare. It occurs more in the occipital, parietal and frontal bone. Rarely has malignant degeneration. The ideal treatment is complete excision and courses with good prognosis. The authors describe a case of giant intradiploic epidermoid cyst located in the occipital bone, associated with cerebellar syndrome. Total resection was performed with good prognosis.

Cisto Epidermoide Intradiploico Gigante Localizado na Fossa Posterior