There are various malformations of retinal vessels. Some of them are associated with cerebral vascular anomalies. This report is given on a girl 6 years of age with a retinal racemose angioma. The clinical examination showed a unilateral racemose angioma combined with retinachorioidal anastomosis and a hemifacial vascular malformation. We found teleangiectasia in this area. The ophthalmoscopy demonstrated a blurred border of optic disc on the right eye with forward protrusion of the disc about + 2.0 dioptres. Large racemose lesions may be localized to the optic nerve and may involve segmental areas of the macula. The ultrasound showed a papillary stasis with highly reflective structures. The ultrasonic patterns are interpreted as a cholesterosis or fibrovascular tissue. This findings may belong to the rare Wyburn-Mason syndrome. Acute neurological symptoms by similar lesions the midbrain and hemorrhages during extraction of teeth are reported in literature.