[A rapidly progressive orbital tumor in a seven-year-old girl revealing a primitive systemic vasculitis]. / Une tumeur orbitaire rapidement évolutive chez une fillette de 7 ans révélant une vascularite systémique primitive.

Orbital tumor lesions in pediatric population encompass a wide range of pathological processes, which are very different in etiology and prognosis. They usually require an emergency in histological diagnosis because of their quick growth. Beside malignant and benign tumors, the inflammatory pseudo-tumors group included dysimmune orbital involvement secondary to systemic vasculitis such as granulomatosis with polyangiitis (ex-Wegener's granulomatosis). We report the case of a seven-year-old girl suffering from a severe primitive ANCA vasculitis, revealed by an orbital mass.