• Cavernous hemangioma of the retina is recognized as a distinct clinical entity. Although neurocutaneous findings are sometimes associated with these retinal tumors, in most of the cases reported to date, systemic features have not been described. This report describes two patients with cavernous hemangioma of the retina. One had neurologic symptoms and signs that were highly suggestive of a hemangioma of the brain stem, and the other had major congenital cardiovascular anomalies. During a five-year interval, the second patient had spontaneous incomplete sclerosis of the retinal hemangioma.