Retinoblastoma is a common pediatric eye malignant tumor, it not only seriously affects children’s eyesight, but also endangers their lives. With the rapid development of biological technology, some breakthroughs have been made in retinoblastoma biological research. It has an important clinical significance to explore the pathogenesis of retinoblastoma in order to inhibit tumor growth and metastasis and improve the survival rate of children. Now the pathogenesis of retinoblastoma research is summarized.