This gene encodes bestrophin-1, an integral transmembrane protein [1], and its variants can result in at least five clinically distinct retinal degenerative diseases: BVMD, adult-onset vitelliform macular dystrophy (OMIM 153700), autosomal dominant vitreoretinochoroidopathy (ADVIRC, OMIM 193220), retinitis pigmentosa (OMIM 613194), and autosomal recessive bestrophinopathy (OMIM 611809) [1–5]. The gene discussed is BEST1; the disease is adult-onset foveomacular vitelliform dystrophy.