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Maffucci's syndrome is a rare, congenital mesenchymal dysplasia characterized by multiple enchondromata and hemangimata, both of which may undergo malignant degeneration. Intracranial involvement is uncommon. A literature review yielded only six cases of Maffucci's syndrome with intracranial chondrosarcoma and two cases of Ollier's disease (enchondromata alone) with intracranial chondrosarcoma. We report the seventh case of Maffucci's syndrome in a patient with a very large and extensive skull base chondrosarcoma requiring staged operations for removal.