Mutations in the gene BEST1 cause five clinically recognized retinal degenerative eye diseases in man: Best vitelliform macular dystrophy (BVMD)1,2, adult onset vitelliform macular dystrophy (AVMD)3, autosomal recessive bestrophinopathy (ARB)4, autosomal dominant vitreoretinochoroidopathy (ADVIRC)5, and retinitis pigmentosa 50 (RP50)6,7. Here, BEST1 is linked to autosomal dominant vitreoretinochoroidopathy.