Iridocorneal endothelial (ICE) syndrome is a group of ocular conditions characterized by corneal proliferative endotheliopathy in which secondary corneal edema, peripheral anterior synechiae, and abnormalities of the iris stroma are the common features. The etiology remains unclear, but may be related to viral infection with Herpes simplex or Epstein-Barr virus. The pathogenesis of the ICE syndrome is believed to result from an abnormality of the corneal endothelial cells (causing corneal edema), with secondary spreading of the cells over the trabecular meshwork region (causing anterior synechiae and elevated intraocular pressure [IOP]) and across the surface of the iris (responsible for the formation of iris holes, pupillary distortion, and iris noduli). The disease complex, which includes essential iris atrophy, Chandler's syndrome, and iris nevus (Cogan-Reese) syndrome, is almost always unilateral, nonfamilial, and typically occurs in females during young adulthood. ICE syndrome is commonly progressive and frequently complicated by secondary glaucoma and corneal decompensation. In Chandler's syndrome, iris changes are less pronounced and corneal edema more frequent than in essential iris atrophy or Cogan-Reese syndrome. Glaucoma associated with ICE syndrome is often difficult to manage and is usually treated with medications and/or filtering surgery. Glaucoma filtering surgery is usually successful when done early, but may fail due to endothelialization of the fistula by the abnormal corneal endothelium.