X-linked retinoschisis is the most common cause of juvenile retinal degeneration in males usually presenting between age 5 and 10 years, and resulting in decreased visual acuity during childhood and adolescence. Vision after that period generally stabilizes at 20/60-20/120, although progressive visual deterioration often occurs later in life. The disorder is characterized by splitting of the nerve fiber layer in the retina. Eye findings include macular schisis, often in a spoke-like pattern; peripheral (usually inferotemporal) schisis in about 50% of subjects; “vitreous veils”; and a decreased b-wave with an intact a-wave on electroretinogram (ERG). The prevalence is estimated to be between 1/5,000 and 1/25,000 males. Although distinct from retinal detachment, retinoschisis may eventually lead to detachment of the retina or retinal atrophy resulting in blindness.