Background: Familial exudative vitreoretinopathy is an uncommon heredodegenerative disease with different types of inheritance and degrees of severity. Methods: Findings in five affected members of a family in which the X-linked mode of inheritance of familial exudative vitreoretinopathy is present are discussed. Results: Patients presented with characteristic retinal exudative tractional detachment, temporal retinal vessel traction, capillary dilatation, neovascularization, and vitreous veils. Conclusion: A review of the 21 cases of X-linked familial exudative vitreoretinopathy reported to date indicates a worse outcome in this mode of inheritance than in cases of autosomal dominant inheritance.