Familial exudative vitreoretinopathy (FEVR) is an autosomal dominant inherited disease. The developmental abnormality of retinal vessels is the basis of various lesions in FEVR. 11 affected members (21 eyes) from 5 families with FEVR were studied. The emphasis was placed on describing the changes of fundus and fundus fluorescein angiography of the disease. Argon laser photocoagulation was performed on 8 patients (14 eyes) and they were followed for 5 approximately 14 months. The diagnosis, differential diagnosis, complications and treatment of FEVR were discussed with some materials in this paper.