A survey of the present state of developments concerning prevention and therapy of hereditary retinal diseases is given. In recent years the techniques of molecular genetics have led to a rapid identification of a great number of cloned or mapped genes responsible for such diseases. Moreover, further progress has been made in the understanding of disease mechanisms by describing the defective proteins and their altered metabolism. The most recent preventive and therapeutic strategies including gene therapy, dietary regimens, anti-apoptotic agents, transplantation and retinal prosthesis are discussed.