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]. This evidence concerns the gene BEST1 and autosomal recessive bestrophinopathy.