The records of 66 patients with the iridocorneal endothelial (ICE) syndrome were retrospectively reviewed. Glaucoma occurred in 33 (50%) of the patients with ICE syndrome and was most common in the variants in which abnormal cells involve the entire posterior corneal surface (disseminated ICE and total ICE). Of 25 patients with glaucoma, the diagnosis of ICE syndrome was overlooked initially in 17 (68%). Medical glaucoma treatment was generally ineffective. Of 22 patients (88%) who underwent surgery (typically trabeculectomy), 10 (45%) required more than one procedure. The success rates of first operations at 1 and 5 years were 60% and 21%, and those of second and third operations at 1 year were 20% and 17%. During the course of their management, 11 patients (44%) developed visual field loss. The diagnosis of ICE syndrome should be considered in younger patients with unilateral glaucoma and confirmed by specular microscopy. Management of glaucoma due to ICE syndrome is surgical, and means of improving its long-term success need to be addressed.