Many vitelliform macular diseases have been described through the years. Some arguments still exist about the appropriate terminology, the classification criteria and the etiopathogenesis. The alterations of the retinal pigment epithelium, the lipofuscin storage and the role of the choriocapillaris are being investigated. The aim of our study was to describe indocyanine angiographic features in Best's disease and pattern distrophies. Twenty-one eyes of 14 patients underwent indocyanine and fluorescein angiography. The indocyanine angiogram showed hypofluorescent and hyperfluorescent areas. The possible etiopathogenesis of the lesions includes a role of the retinal pigment epithelium.