Iridocorneal endothelial syndrome(ICES)is a rare ocular disease characterized by abnormal structure and proliferation of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma and iris atrophy. ICES often occurs in young women, and most of them are monocular. Its pathogenesis is still unclear, the symptoms are various, and the blindness rate is high. The disease is difficult to diagnose, and there is no ideal treatment. The purpose of this article is to review the literature on the characteristics, diagnosis and treatment of ICES in order to help the diagnosis and treatment of the disease.