X-linked juvenile retinoschisis (XJR) is a hereditary disease described as vitreoretinal dystrophy, which is characterized by bilateral detachment of neurosensory retina in macular area and periphery. The schisis can occur in different layers of neurosensory retina, most commonly in the nerve fiber layer, inner nuclear layer, outer nuclear layer and outer plexiform layer. X-linked juvenile retinoschisis is a rare genetic disease, with prevalence that ranges from 1 case per 5,000 to 1 case per 25,000 populations. The main cause is mutation of XLRS1 gene, which is located on X chromosome. Differential diagnosis includes niacin overdose, oculocutaneous albinism and X-linked albinism, chemotherapy (toxic maculopathy), retinitis pigmentosa (Golmann-Favre and other forms), and Epiretinal membrane and vitreomacular traction syndrome. Clinical examination of macula and retinal periphery is the first step toward diagnosis. Fundoscopic examination can reveal stellate-pattern microcysts in the center or retinal periphery. Fundus photography and optical coherence tomography (OCT) can help in diagnosis of XJR.