Juvenile xanthogranuloma is a self-limited skin disorder of young chil
dren that uncommonly affects the eye. Juvenile xanthogranuloma has bee
n described in adults, but reported intraocular involvement is extreme
ly rare. We report a case of juvenile xanthogranuloma diagnosed in a 2
5-year-old man who was seen with nontraumatic hyphema and iridocycliti
s. Diagnosis was made from a biopsy specimen obtained from a suspiciou
s skin lesion. Topical and systemic steroids, radiation therapy, and f
inally immunosuppression were required to eliminate the iris tumor cli
nically and resolve the patient's recurrent symptoms.