Autosomal recessive ocular syndrome associated with microphthalmia, retinitis pigmentosa, retinoschisis, and OND has been described by different genes and mutations [MFRP (19), PRSS56 (20), MYPF (21, 22), CRB1, BEST1 (21), and so on]. This evidence concerns the gene BEST1 and microphthalmia.