Juvenile X-linked retinoschisis (JXR) is a vitreoretinal dystrophy affecting males only. The macular and peripheral retinal alterations associated with this disorder are very specific. Initially, a stellate-shaped foveal schisis forms; as the patient becomes older, chronic retinal pigment epithelial disturbance leads to macular degeneration. Peripheral retinoschisis may also occur, but is only identified in about half of all patients with JXR. These changes, which represent degenerating vitreous, may be complicated by retinal breaks due to the thin layer of the schisis. This paper presents five cases of JXR in patients ranging from ages 4-70 years and discusses and photodocuments the progression of related retinal changes. A table of differential diagnoses is also included.