Solitary orbital involvement with juvenile xanthogranuloma

A newborn girl was noted to have a smooth superonasal orbital and preseptal mass at the time of birth. The mass enlarged slightly over 3 months and a biopsy revealed histiocytes and Touton giant cells, compatible with juvenile xanthogranuloma. The infant had no other manifestations of juvenile xanthogranuloma. The mass subsided after systemic corticosteroid therapy was administered. Solitary orbital involvement with juvenile xanthogranuloma is exceedingly rare and most of these patients are found to lack the typical cutaneous lesions. Observation, oral prednisone, or low-dose radiotherapy are recommended forms of treatment.