Persistent Fetal Vasculature (PFV), previously known as Persistent hyperplastic primary vitreous (PHPV), is a rare congenital developmental malformation of the eye, caused by the failure of regression of the primary vitreous. It can occur in isolation, or in association with other ocular disorders and rarely as a part of systemic disorder. Although genes responsible for PFV are unknown, the lack of inheritance suggests that idiopathic, isolated, sporadic mutations may be responsible for the disease; a developmental defect caused by environmental factors during embryogenesis rather than genetic factors. Characteristic features include microphthalmic eye, white vascularised retrolental tissue with or without a persistent hyaloid artery, centrally dragged ciliary processes, an anteriorly shifted and (or) swollen lens, and varying degrees of lenticular opacification. Radiological investigations (ultrasound, computerised tomography, magnetic resonance imaging) aid in the diagnosis and differentiation from other causes of leukocoria like retinoblastoma. This case report discusses an 8 month old male child with bilateral Persistent Fetal Vasculature.

Keywords: Persistent Fetal Vasculature, leukocoria, microphthalmos.