Abstract: Familial exudative vitreoretinopathy (FEVR), a rare hereditary vitreoretinal abnormality, characterized by insufficient vascular development is a leading cause of visual impairment and even blindness in people under 30 years old. It is a lifelong disease with obvious clinical heterogeneity and unpredictable progression. Although significant presentations may not occur in the early stages, the characteristic clinical features show up with progression, which includes avascular peripheral retina, subretinal exudation, peripheral retinal neovascularization, retinal fold, macular dragging, and even retinal detachment resulting in blindness finally. In order to prevent or stabilize the disease, preserve visual function, and further improve the quality of life, it is of significance to pay close attention to identifying clinical stages of FEVR according to ocular manifestations and wide-field angiography, and developing appropriate clinical management strategies. Early identification, regular follow-up and genetic counseling should be performed in clinical practice. Photocoagulation, cryotherapy and anti-vascular endothelial growth factor therapy can be applied in early stage to control exudation and neovascularization. Scleral buckling and vitrectomy can be used in late stage to prevent unfavourable prognosis. In addition, for pediatric and adolescent patients with FEVR, it is indispensable of functional vision training and psychological rehabilitation as well .