The first case of retinal involvement in hereditary hemorrhagic telangiectasia in the American ophthalmic literature shows a marked similarity to diabetic retinopathy. In a patient with the ophthalmoscopic findings of retinal angiopathy and normal glucose tolerance tests, Rendu-Osler-Weber disease, although a rarity, should be considered. The diagnosis can be confirmed by family history of bleeding diathesis and careful inspection of skin and mucous membranes. Cautious photocoagulation therapy merits consideration in such cases.