Juvenile xanthogranuloma of the orbit in an adult

We present a case of juvenile xanthogranuloma (JXG) with unilateral involve ment of the orbit and eyelid and proptosis, histologically confirmed in a 3 2-year-old man with a 1-year history of a pansinusitis and dacryoadenitis w ith rhinitis. Nine months later an infiltration of the anterior upper part of the right orbit and right eyelid appeared. Computed tomography scan and magnetic resonance imaging studies confirmed the presence of pansinusitis a nd infiltration. The patient underwent a blepharoplasty and excision of the infiltrated tissues of the orbit, eyelid, and levator muscle. Hematoxylin- eosin and immunohistochemical studies revealed features consistent with a d iagnosis of JXG (Touton giant cells). JXG, a non-Langerhans'-type benign pr oliferation, is a rare condition in adulthood.