Retinal detachment occurred in 36 (20%) of 180 eyes of 90 individuals with familial exudative vitreoretinopathy (FEVR) from 17 families. FEVR can give rise to very different types of retinal detachment, such as rhegmatogenous retinal detachment, falciform retinal folds, PVR with formation of a retrolental membrane and exudative retinal detachment. All but one retinal detachment occurred before the age of 30. Traction from vitreous membranes seems to be the most important cause of retinal detachment; atrophy of the peripheral retina may contribute to the formation of retinal breaks. Subretinal exudation is a rare cause of FEVR retinal detachment.