Mutations in BEST1 have since been found to be causative of other distinctive retinal dystrophies, including autosomal dominant vitreoretinochoroidopathy (ADVIRC; OMIM 193220) [3–7], autosomal recessive bestrophinopathy (ARB; OMIM 611809) [8–10], adult vitelliform macular dystrophy [11,12], and more recently, what was described as “concentric retinitis pigmentosa” [13]. Here, BEST1 is linked to autosomal dominant vitreoretinochoroidopathy.