A range of posterior segment abnormalities, especially anomalies of the optic papilla, coloboma, and persistent hyperplastic primary vitreous are associated with the high risk to develop a retinal detachment. Most of these retinal detachments develop in the first and second decade of life. In most cases of retinal detachment caused by malformations, vitreoretinal surgery is indicated. Results of surgery depend on the nature of malformations. In spite of an anatomic re-attachment of the retina, in some eyes the functional results are limited. Especially in cases of severe PHPV, the aim of vitrectomy is only to prevent complications. Vitreoretinal surgery should not be performed in eyes with no light perception, without papillary light reflex, and/or with unrecordable visually evoked potential.