Sarcomas infrequently develop in osseous sites of fibrous dysplasia. We rep
ort a patient with Mazabraud's syndrome (polyostotic fibrous dysplasia and
soft tissue myxomas) complicated by the development of osteogenic sarcoma i
n a bone affected by fibrous dysplasia. This is the third case of osteosarc
oma within the small population of reported patients with Mazabraud's syndr
ome. There may be an increased incidence of malignant transformation in the
se individuals' dysplastic bones above that associated with patients suffer
ing from fibrous dysplasia alone.