Nevoxantho-endothelioma is a benign nevus with xanthomatous changes. It appears at birth or shortly thereafter. The process may occasionally be mistaken for sarcoma, but usually follows a benign course with crops of new lesions which eventually tend toward spontaneous involution. This case demonstrates that the eye can be involved by lesions having the same histologic structure as those of the skin. Because of the favorable course of patients suffering from this disease and the realization that visceral lesions may occur in nevoxantho-endothelioma, early diagnosis insures an accurate favorable prognosis.