Juvenile xanthogranuloma (JXG) is a disease of unknown etiology and pathogenesis which was first recognized by dermatologists. The ocular complications provide the primary concern and notably affect the iris, producing spontaneous hemorrhages in the anterior chamber. Secondary glaucoma and blindness are real threats unless the condition is recognized in the early stages and satisfactory treatment instituted. Other ocular manifestations include epibulbar lesions with corneal involvement and proptosis due to orbital lesions of JXG.