3 cases with various degrees of racemose hemangiomata of the retinal vessels are described, in whom the optic nerve showed primary involvement. The vascular malformation ranged from multiple arteriovenous shunts (2 cases) to marked vascular tortuosity, and the optic nerve showed involvement that varied from severe hypoplasia with blindness, to normal appearance with campimetric enlargement of the blind spot. The combined malformation must be placed at the 110-mm embryonic stage, and the tissue primarily involved is the vascular tree branching from the developing central retinal artery at the end of the 4th month. The vascular anomaly, in turn, causes underdevelopment of optic nerve tissue and involvement of the bony orbit.