X-linked Retinoschisis is a bilateral retinal disease with a recessive X-linked inheritance, characterized by a macular involvement. Maculopathy consists of bilateral star-shaped microcystic changes. Peripheral retina is sometimes involved with schisis. In two years, were examined 10 eyes of 5 male patients (age range 20-25 years old) with X-linked Juvenile Retinoschisis and progressive reduction of visual acuity. Macular alterations were classified as: Typical foveal schisis; Altered foveal reflection; Lines of pigmentary demarcation; Retinal pigmentary epithelium atrophy; Hole in retinal internal layer. OCT images showed a separation of neurosensorial retina that coincided with classical histopathological findings. Examination results were correlated with clinical examination and compared with ERG findings. OCT images underline an increase of retinal thickness with a separation in the nerve fiber layer in the foveal space.