We examined 69 members of two caucasian families with an autosomal dominant form of central areolar choroidal dystrophy using ophthalmoscopy and fundus photography. In five members who had a good visual acuity macular lesions were found. In three of them retinal function tests were performed, which turned out to be normal. These 5 patients underwent fluorescein angiography. Early symptoms of central areolar choroidal dystrophy are small parafoveal hyperfluorescent areas due to retinal pigmentepithelium loss and areas of pigment mottling in the macula. Until now the early lesions of central areolar choroidal dystrophy in patients with a good visual acuity in both eyes have not been described clearly.