At age 26 years a woman who had been blind in the left eye from birth had visual acuity of R.E.: 6/18 (20/60); L.E.: 6/120 (20/400), with 40 prism diopters of left exotropia. The left eye showed a decreased anteroposterior diameter of the globe and a complete retinal detachment behind a cataractous lens. The right eye had a posterior subcapsular lens opacity severe vitreous fibrosis, dragging of the optic disk, and intraretinal and subretinal exudation with a fibrovascular mass in the temporal retina. The patient's 3-year-old daughter had 45 to 50 prism diopters of exotropia, a pendular nystagmus with intraretinal and subretinal dragging of each disk, and early degeneration and band formation in the periphery of each fundus.