Dominant NR2E3 gene mutation has been associated with RP phenotype [17–20] and to date, the phenotype has only been described in five European [17, 18, 20] and four North American [19] families characterized by presenting RP, but not to other phenotypes associated to NR2E3 gene recessive mutations [16, 17]. Here, NR2E3 is linked to retinitis pigmentosa 1.