Iridocorneal endothelial syndrome(ICE syndrome)is one of the relatively rare clinical ocular diseases, of which pathogenesis is not clear. The symptoms are complicated, and the risk of blindness is very high. Patients often see a doctor due to glaucoma, visual loss, eye pain and abnormalities of the iris. Until recently there is not any ideal method to treat the syndrome. In clinical practice, doctors only have to treat the complications of this disease, using medicine or surgeries. This article will review the recent literature on the treatment of ICE syndrome, at the same time to identify the disease prone to misdiagnosis, to provide help for the treatment of ICE syndrome.