Persistent hyperplastic primary vitreous (PHPV) is a disorder of ocular development which occurs due to incomplete regression of the embryonic vitreous and hyaloid vasculature. Most cases of PHPV are sporadic and unilateral. We report a case of PHPV in a 5-year-old male who presented with complaint of decreasing eye size. Grey-scale ultrasonography (US) evaluation revealed an hypoechogenic band in the posterior segment of the left globe extending from the posterior surface of the lens capsule to the optic disc. Also magnetic resonance imaging (MRI) findings suggested the diagnosis of persistent hyperplastic primary vitreous.