Nevoxanthoendotheliomas (and juvenile xanthogranulomas) are very rare benign skin diseases which occur in infants. An ocular manifestation, mainly in the iris, has been found in approximately. 10% of the cases, The clinical picture is characterized by a recurrent unilateral hemorrhage of the anterior chamber, which is the reason for seeing an ophthalmologist. The clinical diagnosis of juvenile xanthogranuloma is based on the characteristic iris changes - heterochromia, circumscribed vascularized tumor, or diffuse thickening of the iris. Sometimes elevated intraocular pressure is also found. In combination with a mild local steroid, radiotherapy with 300 to 400 cGy should be started immediately in order to prevent a sometimes uncontrollable secondary glaucoma. The good results achieved in 5 of the authors' own cases underline the value of low-dose radiotherapy in this disease: the affected eye was healed in all 5 cases.