BEST1 variants are inherited in both autosomal dominant and recessive manners and are linked to five distinct phenotypes referred to as “bestrophinopathies”: Best vitelliform macular dystrophy (BVMD) (OMIM #153700),1,2 adult-onset vitelliform macular dystrophy (AVMD) (OMIM #153700),12,13 autosomal recessive bestrophinopathy (ARB) (OMIM #611809),9 autosomal dominant vitreoretinochoroidopathy (ADVIRC) (OMIM #193220),14 and retinitis pigmentosa (RP) (OMIM #613194).15 The gene discussed is BEST1; the disease is autosomal dominant vitreoretinochoroidopathy.