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Fourth ventricle epidermoid cysts or pearly tumors are uncommon lesions which arise from epithelial remnants at the time of neural tube closure. They are benign extracerebral intra-dural lesions accounting for <2% of intracranial tumors out of which only 5-18% may occur in the fourth ventricle. Epidermoid cysts of the fourth ventricle occur very rarely, with few cases reported in the literature. We present a case of a pathological proven less common fourth ventricle epidermoid cyst.