During development, the fetal vasculature nourishes the developing lens and retina, and it subsequently regresses after the formation of the retinal vessels. Persistent fetal vasculature (PFV) occurs as a result of a failure of fetal ocular vasculature to undergo normal programmed involution, which leads to blindness or serious loss of vision. Persistent fetal vasculature is responsible for as much as 5% of childhood blindness in western countries. The regulatory mechanisms responsible for fetal vascular regress remain obscure, as do the underlying causes of the failure of regression. Because of recent advancements in microinvasive surgical techniques, the early treatment of PFV has become safer and more effective, thus paving the way for the development of a future new treatment strategy. In this review, clinical and imaging manifestations of PFV and the progress in the treatment of PFV are highlighted.