Primary vitreous hyperplasia syndrome (persistent hyperplastic primary vitreous) is a congenital anomaly of the vitreous caused by the failure of the primitive vitreous and vitreous vessels to disappear during the embryonic period and continue to proliferate. According to the ocular condition, there are three categories: 1, simple anterior PHPV (about 25%): including shallow anterior chamber, elongated ciliary process, glaucoma, coarse iris vessels, posterior lens membrane, posterior fibrous sheath, cataract. 2.Simple posterior PHPV (about 12%): vitreous tip, vitreous fasciculus, anterior retina, retinal folds, retinal detachment, abnormal development of macula and optic disc. 3. Coexistence of both types (about 63%): the most common clinical condition. PHPV is extremely rare, most of them have no clear etiology, most of them have monocular onset (90%), it is more common in males, mostly seen in full-term infants or children, no abnormal history during maternal pregnancy, and it is one of a group of childhood white pupil disease. To date, the cause of PHPV has not been fully elucidated, and the currently accepted cause is the failure of the primitive vitreous humor to degenerate at 7-8 months of embryonic life, but the abnormal proliferation. The clinical symptoms are shallow anterior chamber, glaucoma, white pupil, posterior crystalline fibrovascular membrane, intraocular hemorrhage, retinal folds and retinal detachment. the progression of PHPV is mainly the aggravation of cataract, progressive shallowing of anterior chamber causing angle-closure glaucoma, intraocular hemorrhage, pulling ciliary protrusion and peripheral retinal detachment leading to eye atrophy. Early surgical treatment is needed to remove the risk factors that cause these crosses, to stop their progression, to restore useful vision and to preserve the eye. Early diagnosis and early treatment of PHPV allows the child to be treated as early as possible and prevents the development of amblyopia. As seen in the literature, the timing of surgery should be chosen within the first 3 months of life as early as 2 months.