Genetic ocular diseases are inherited Mendelian conditions (prevalence 1/1000) in which any tissue of the eye could be involved (cornea, lens, iridocomeal angle, vitrous, retina, choroid, sclera). More than 200 genes are responsible for inherited retinal dystrophies and even more genes remain to be identified. These genes belong to many metabolisms essential to the photoreceptor function. Gene therapy and retinal prosthesis are the two most promising therapeutic strategies currently in clinical trials which are expected to provide visual improvement in short term.