<h4>Purpose</h4>To report a rare case of spontaneous vitreous and intraretinal hemorrhage in a patient with juvenile X-linked retinoschisis which was managed conservatively.<h4>Methods</h4>Single patient case report.<h4>Introduction</h4>Juvenile X-linked retinoschisis (JXLR) most often occurs as a result of a genetic defect in the retinoschisin (RS1) gene, causing a separation between the ganglion cell layer and the nerve fiber layer. The gene discussed is RS1; the disease is X-linked retinoschisis.