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To assess the anatomical and functional results of vitreoretinal surgery in patients with advanced familial exudative vitreoretinopathy (FEVR).Retrospective analysis of data for 10 eyes of 9 patients with advanced FEVR who underwent pars plana vitrectomy from March 1997 to May 2015 and had a follow-up of at least 12 months. The primary outcomes were final visual acuity (VA) and anatomical success.The average age at the time of the surgery was 10.1±6.5 years (range 2 months–18 years). The mean follow-up period was 58.4±75.1 months. The male-to-female ratio was 7/2. The mean number of vitreoretinal operations was 1.5. At the last visit, 7 (70%) eyes had complete or partial retinal attachment. Preoperatively, the mean Snellen VA was 20/4000 (n=8), and in the remaining 2 (20%) eyes it was recorded as ‘central, steady and maintained’. At final examination, the mean Snellen VA was 20/330 (n=7), and in the remaining 3 (30%) eyes it was recorded as light perception. At the final visit, 5 eyes (50%) had improved VA, 2 eyes (20%) showed stabilisation, and 3 eyes (30%) with total retinal detachment had a decrease in VA. No progression to glaucoma was observed and no enucleation was necessary.Advanced FEVR in young children tends to be more aggressive, leading to severe complications. These cases are challenging and require special consideration. Despite surgery, disease-related complications remain high. However, surgical intervention for advanced FEVR might be of benefit in helping to preserve vision.