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To evaluate the clinical outcome of penetrating keratoplasty (PK) in iridocorneal endothelial (ICE) syndrome.Clinical charts of patients who underwent penetrating keratoplasty for ICE syndrome between 1985 and 1999 were reviewed retrospectively. Glaucoma control, best corrected visual acuity pre-and post-PK, graft clarity, graft rejection episodes, improvement in pain, and additional procedures were analyzed.Fourteen cases were reviewed with an average follow-up of 58 months after PK. Initial grafts failed in seven patients (50%), in six cases because of rejection, and one owing to endothelial failure without signs of rejection. Repeat PKs were performed in six patients. At final follow-up, 12 grafts were clear. Glaucoma was controlled pre-and post-PK (average intraocular pressure, 16 mmHg for both eyes). Pre-PK, eight patients were using glaucoma medicines and nine had had glaucoma surgery. At the end of the follow-up, seven patients were using glaucoma medicines; six patients required glaucoma surgery after their initial PK. At the final follow-up visit, visual acuity in three patients (21%) was 20/40 or better, it ranged from 20/50 to 20/100 in four patients (29%) and 20/200 to 20/400 in five patients (36%), and in two patients with failed grafts (14%) it was counting fingers or worse.Clear grafts were achieved in 12 cases, although six patients (43%) underwent repeat PKs. All patients had glaucoma, which was controlled before and after PK by medical treatment and surgical procedures. Favorable outcomes can be achieved in patients with ICE syndrome but may require multiple corneal and glaucoma procedures.