Rare case of non-typical bilateral persistent hyperplastic primary vitreous

Persistent hyperplastic primary vitreous (PHPV) is a rare congenital ophthalmic developmental disorder that occurs as a result of incomplete regression of the embryonic hyaloid vasculature. Usually, it presents unilaterally but it can also present bilaterally which is rare. It conventionally manifests with leukocoria, microphthalmia and retinal dysplasia which has a poor visual prognosis. Nevertheless, there is a paucity of awareness on instances of PHPV in teenage without typical manifestations. This article dispenses the presentation of an atypical PHPV and reviews the contemporary comprehension for this diagnosis. It describes a 12-year-old boy with bilateral PHPV who came for routine ophthalmic examination with no significant history which discriminates this presentation from typical PHPV. It is imperative to raise perception of congenital ocular diseases and the significance of dilated fundoscopy and provide timely management.