Iridocorneal endothelial(ICE)syndrome presents a group of ocular pathologies mostly characterized by corneal endothelial abnormalities and iris atrophy. Iridocorneal endothelial syndrome is typically a unilateral and generally not involving genetic condition, frequently affecting women in early to middle adulthood. There is no treatment for absence of symptoms in early stages. When complicated with corneal edema and secondary glaucoma, treatment is mainly symptomatic. In this group of diseases, there are many similarities and some differences in their pathogenesis, clinical manifestations and treatment. So far no universal information on these features has been published. Recent clinical studies on iridocorneal endothelial syndrome are reviewed in this paper.