Bestrophinopathies are a group of inherited retinal diseases caused by mutations in the BEST1 gene [1,2] which include classical Best vitelliform macular dystrophy (BVMD) [3,4], adult-onset vitelliform macular dystrophy (AVMD), autosomal dominant vitreoretinochoroidopathy (ADVIRC), autosomal recessive bestrophinopathy (ARB) [5] and retinitis pigmentosa (RP) [6,7]. The gene discussed is BEST1; the disease is adult-onset foveomacular vitelliform dystrophy.