Bilateral persistent hyperplastic primary vitreous (PHPV) is an uncommon entity which usually presents as leukocoria, microphthalmia, and cataract.[1] It is a developmental disorder of the eye which occurs due to abnormal persistence of fetal intraocular vessels in the anterior or posterior segments of the eye.[2] Primary vitreous forms around 7th week of intragestation life and starts involuting around 20th week and nearly always disappears at the time of birth. Failure of regression of primary vitreous results in many of the abnormalities seen in PHPV.[3] In these cases, the child usually presents due to poor vision and white reflex.[1] The entity often presents a diagnostic challenge as it is often confused with retinoblastoma.

Bilateral persistent hyperplastic primary vitreous (PHPV) is an uncommon entity which usually presents as leukocoria, microphthalmia, and cataract.[1] It is a developmental disorder of the eye which occurs due to abnormal persistence of fetal intraocular vessels in the anterior or posterior segm...