Best vitelliform macular dystrophy (BVMD) is autosomal dominant juvenile onset maculopathy, which is associated with a mutation in human BEST1 gene.[1,2] hBEST1 gene encodes bestrophin-1 (Best1) protein, which is expressed basolaterally in retinal pigment epithelium (RPE) [3] and in astrocytes.[4] BVMD involves several stages and leads to loss of central vision. This evidence concerns the gene BEST1 and Best vitelliform macular dystrophy.