Retinoblastoma is the malignant intraocular tumor most commonly happened on children, especially the children under 3 years old. It may lead to blindness, eyeball removal and even death, which severely threaten the safety and quality of children’s life. Exploring the origin of tumor cells is conducive to the reveal of the pathogenesis as well as the guidance on diagnosis, classification and treatment of retinoblastoma. Based on the studies on retinal development, three theories regarding the cell origin of retinoblastoma, namely the origin of retinal precursor cells, the origin of cone cells and the origin of horizontal cells or Müller glia cells have lately been proposed. This article reviews and summarizes the research of the origin cells in retinoblastoma.