The Notch signaling pathway is an evolutionarily highly conserved signaling pathway that exists in a wide variety of tissue cells and plays an important role in embryonic development, cell proliferation, differentiation and apoptosis, as well as in the maintenance of multicellular biosynthesis, angiogenesis, and the development of various cancers. In recent years, it has been found that the Notch signaling pathway is abnormally activated in cataract, uveitis and other ophthalmic diseases, regulating the process of ocular disease development. This article provides a brief review of the regulatory role of the Notch signaling pathway in the development of ophthalmic diseases.