Juvenile xanthogranuloma is a self-limited skin disorder of young children that uncommonly affects the eye. Juvenile xanthogranuloma has been described in adults, but reported intraocular involvement is extremely rare. We report a case of juvenile xanthogranuloma diagnosed in a 25-year-old man who was seen with nontraumatic hyphema and iridocyclitis. Diagnosis was made from a biopsy specimen obtained from a suspicious skin lesion. Topical and systemic steroids, radiation therapy, and finally immunosupression were required to eliminate the iris tumor clinically and resolve the patient's recurrent symptoms.