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To report the clinical characteristics of the affected and fellow eyes in patients with familial exudative vitreoretinopathy–associated rhegmatogenous retinal detachment (FEVR-RRD).This was a retrospective observational case series. The affected and fellow eyes were given complete examinations. The age, sex, clinical features of the affected eyes, and varied abnormalities of the fellow eyes were analyzed.Forty-three patients with FEVR-RRD were included. The average age was 21.8 ± 10.9 years, and the males (19.9 ± 9.6) were younger than the females (28.1 ± 1.2). Retinal round holes were noted in 32 (71.1%) eyes. In addition, horseshoe retinal tears and giant retinal tears were present in 10 (22.2%) eyes and 3 (6.7%) eyes. Subretinal fibrosis was seen in 20 (44.4%) eyes, and choroidal detachment was shown in 6 (13.3%) of the eyes. A high prevalence of abnormalities was noted in the undetached fellow eyes in the peripheral retina, including vascular leakage in fundus fluorescein angiography (75.6%), lattice degeneration (53.7%), and vitreous traction (51.2%).Male patients with FEVR-RRD experience an earlier onset than females in our series. Retinal tears, even giant tears, could be responsible for FEVR-RRD. The fellow eyes of FEVR-RRD patients were characterized by predetachment changes, which need both lifelong monitoring and timely vision-saving intervention.