Bilateral persistent hyperplastic primary vitreous (
PHPV) is a rare ocular disorder. Its clinical manifestations include bilateral corneal haziness, microphthalmia, and cataract. It is the second most common cause of leukocoria after retinoblastoma. Most cases of
PHPV are unilateral. The typical imaging features of
PHPV comprise bilateral echogenic masses and a fibrous cord extending from the posterior surface of the lens to the optic disc. In this report, we present a case of bilateral
PHPV in an infant who presented with bilateral corneal haziness and watery discharge. A detailed ocular examination and knowledge about its features on imaging can lead to a timely and accurate diagnosis of the condition.