[Juvenile aggressive palpebral fibromatosis. Apropos of a case]

Juvenile Aggressive Fibromatosis (JAF) is histologically characterized by a fibroblastic cellular proliferation. Even though benign, it is locally invasive and very often recurrent but never induces metastasis; the evolutionary argument is the only one which enables us to differentiate JAF from fibrosarcoma. The selected treatment is extensive surgical excision including healthy tissue with histological control. The prognosis is variable depending upon localization and therapeutic possibilities. We report the case of a seven year-old girl presenting an eyelid localization which is seldom reported. The extraorbital and intracranial extension was fatal in spite of the therapy associating surgery, radiotherapy and chemotherapy. The central nervous system proximity poses a difficult problem and speaks for an early mutilating surgery. However, the small number of published cases does not yet allow codification of treatment.