ObjectiveTo observe and explore the fundus characteristics and fundus fluorescein angiography of familial exudative vitreoretinopathy (FEVR) in different stages. MethodsA total of 15 patients (23 eyes) diagnosed as FEVR in the West China Hospital of Sichuan University from January 2007 to November 2013 were included. Clinical data and reports of fundus exams and fundus fluorescein angiography (FFA) were retrospectively analyzed. ResultsOne eye (4.35%) was classified as stage Ⅰ, 10 eyes (43.48%) were classified as stage Ⅱ, 8 eyes (34.78%) were classified as stage Ⅲ, and 1 eye (4.35%) and 3 eyes (13.04%) were classified as stage Ⅳ and V, respectively. The outcomes of fundus exams showed that the number of peripheral retinal blood vessels increased, and vessels straightened as well as narrowed, especially in the temporal area. FFA showed blood vessels suddenly shut in the equatorial retina and peripheral non-perfusion areas were observed. ConclusionTypical fundus characteristics and fundus fluorescein angiography changes of FEVR can be observed in different stages. Comprehensive fundus exams and family history are helpful to confirm relevant diagnosis.