IntroductionFetal persistent hyperplastic primary vitreous (PHPV) and retinoblastoma (Rb) is rare congenital abnormal development of the vitreous body1,2. According to related manifestations after delivery, PHPV is clinically called “white pupil.” It mainly manifests as vision loss and lens opacity and, in severe cases, as intravitreal hemorrhage and eyeball atrophy. The disease is possibly isolated or associated with congenital syndromes, such as Walker–Warburg syndrome and Norrie disease. Rb is the most common intraocular malignancy of childhood. The role of prenatal ultrasound screening for Rb has been discussed3. Here, we present a case of persistent primary hyperplasia vitreous diagnosed in the second trimester using prenatal ultrasonography. An echogenic band with an irregular surface was detected between the lens and the posterior wall of the left eye. No other system abnormalities were present in the fetus.