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Maffucci's syndrome is a rare, congenital mesodermal dysplasia combined with dyschondroplasia and hemangiomatosis. Enchondromatous involvement of the skull bones is rare in this syndrome. A rare case of Maffucci's syndrome associated with enchondroma at the skull base, left internal carotid artery aneurysm, and goiter is reported. Two other previously reported cases of Maffucci's syndrome with associated aneurysms and the present case suggest that Maffucci's syndrome may be associated with aneurysm.
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