Retinoblastoma is a common eye malignant tumor which has high degree of malignancy.The traditional treatment methods are destructive, and the prognosis is poor. Hypoxia-inducible factor-1α highly express in retinoblastoma and regulate the development and progression of retinoblastoma(by influencing the proliferation and differentiation of tumor cells and involving in the generation of vascular mimicry). This article reviewed the role of hypoxia inducible factor-1α in the development of retinoblastoma which will initiate a new avenue for the treatment of retinoblastoma.