To describe vitreoretinal abnormalities in patients with anterior megalophthalmos (AM) and to report the results of vitreoretinal surgery in these cases. In a retrospective, noncomparative case series, a diagnosis of AM was made according to the following criteria: megalocornea, iris hypoplasia, lens subluxation, cataract formation at an early age, and absence of congenital glaucoma. Vitreous biomicroscopy and funduscopy were performed in all patients. Patient records were also reviewed. Twenty-four patients with AM (12 male, 12 female), aged 4 months to 64 years, were included in this study. Funduscopy was possible in 46 eyes. Vitreous degeneration was the most common abnormal posterior segment finding, present in 34 eyes (73.9%). Nine eyes (19.5%) had optically empty vitreous with strands. Lattice degeneration was seen in five eyes (10.8%). Spontaneous vitreous hemorrhage occurred in three eyes, and peripheral retinal neovascularization was detected in two. Retinal detachment occurred in 18 eyes (37.5%); retinal breaks were detected in eight of these before surgery. Thirteen eyes underwent a total of 18 procedures. Retinal reattachment was finally achieved in 12 of the 13 eyes. Anterior megalophthalmic eyes seem to be affected by a type of vitreoretinopathy predisposing to retinal detachment. Current vitreoretinal surgical techniques usually achieve good anatomic results in these cases.