Persistent fetal vasculature (PFV), also known as persistent hyperplastic primary vitreous (PHPV), is a congenital ocular anomaly, which is common in infants and young children. Due to most children have unilateral occurrence, insidious symptoms and are easily misdiagnosed as simple congenital cataract, the optimum time for treatment is often delayed. Therefore, correct diagnosis and appropriate treatment are particularly significant for the prognosis of PFV children’s visual function. A male child aged 6 years and 6 months with a diagnosis of combined PFV is reported, whose ocular features were congenital cataract and structural dislocation of macula.