The fovea is an anatomical specialization of the vertebrate retina critical for high spatial visual acuity and refined color vision. In mammals, the fovea is present only in humans and primates. It is characterized as a cone photoreceptor-rich region, with a central rod-free avascular zone containing elongated cone photoreceptors and Müller cell processes. A clearer understanding of the molecular mechanisms implicated in foveal development and the consequences of abnormalities of this process is emerging. This will help to explain why phenotypic variability occurs within specific developmental diseases. In addition, although human foveal development begins mid gestation, it continues after birth, thus providing a window of opportunity to remodel the abnormal foveal architecture through pharmacological approaches in young patients otherwise destined for poor vision.