Persistent hyperplastic primary vitreous is also known as persistent fetal vasculature (PFV). It is usually a unilateral phenomenon accompanied by a smaller eye (1,2). The almost uniform incidence of amblyopia means that these cases should be operated on early (3). An additional argument for early surgery is the prevention of long-term traction detachment and pupillary block chamber (4,5). This condition is usually recognized early in life, and the patient should be operated upon if a traction detachment is recognized, the cataract is sufficient to cause visual loss and amblyopia, or there is shallowing of the anterior chamber secondary to pupillary block. The embryological explanation for the syndrome is the lack of regression of the primary vitreous and hyaloid vasculature, although the primary cause is still unclear. Bilateral cases in males are usually associated with Norrie’s syndrome. Norrie’s cases should not be operated on because the retina is dysplastic and the vitreoretinal interface cannot be delineated at surgery.