Retinoblastoma(RB)is the most common intraocular malignant tumor in children, which often occurs in infants and young children. It can damage the eyesight, eyeball and even life-threatening of children. The occurrence and development is closely related to the genome and epigenome. The treatment and prognosis of hereditary RB in all cases depends on the initial stage of the disease, and when the disease is diagnosed and treated early, the survival rate exceeds 95%. The survival rate of RB was less than 50% when extraocular dissemination occurred in late stage. Therefore, the treatment focus on RB is to save the lives of children through early tumor monitoring and prevention. The secondary goal is to preserve globe salvage and to preserve vision to the maximum extent possible. The research on RB genetics is helpful to find specific targets through gene screening, monitoring and search, which is very necessary to improve the survival rate of children and to research targeted therapy.