[Maffucci syndrome and ovarian tumor]

Maffucci's syndrome was first described in 1881 and results of a mesodermic dysembryoplasia, congenital but not hereditary. Pathogenic hypothesis are multiple. This syndrome is characterized by the occurrence of multiple haemangiomas in the soft tissue, and multiple enchondromas of the bones. The association of ovary tumor is however exceptional. Four cases are reported in the literature; we report the fifth case.