Abstract: The development of macular fovea can be divided into four stages: early embryonic stage, late embryonic stage, early stage after postnatal and mature stage. There are many factors which could affect the process of foveal development, such as foveal avascular zone, Müller cells and macular pigment. This process involves the bidirectional movement of inner and outer retinal neurons, involving ganglion cells, inner layer cells, photoreceptors and other cells. Foveal hypoplasia (FH) refers to the absence of both fovea pit and avascular zone. According to OCT images, the severity of FH is divided into 1~4 grades typical and atypical foveal hypoplasia, which is related to albinism, congenital aniridia, isolated central foveal hypoplasia and other diseases. Its pathogenesis is still unknown. (Int Rev Ophthalmol, 2022, 46: 465-470)

Key words: macular fovea hypoplasia