Thus, abnormal functioning of this protein leads to a variety of retinopathies.[1] Autosomal recessive bestrophinopathy (ARB) (OMIM-611809) is a subtype of bestrophinopathy caused by biallelic mutations of the BEST1 gene.[2] Clinical findings in patients with ARB differ from those in patients with Best vitelliform macular dystrophy (BVMD). Here, BEST1 is linked to Best vitelliform macular dystrophy.