X-linked juvenile retinoschisis is an infrequently occurring inherited disease affecting males. Female carriers are phenotypically normal. A stellate or wheel-spoke macular cystic appearance is considered by some to be pathognomonic for the disease in early stages. One half of the cases also have peripheral retinal and vitreal defects. We review the fundus changes, histologic characteristics, visual course and electrophysiologic findings of the disease, and describe an illustrative case and its low vision management.