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To report the long-term outcomes of Boston keratoprosthesis type II implantation in the management of severe ocular surface disease and corneal blindness through a retrospective interventional case series.This retrospective review included medical records of patients who underwent Boston keratoprosthesis type II implantation at the Massachusetts Eye and Ear Infirmary from January 1, 2000 through December 31, 2009. The main outcome measures analyzed were visual acuity, keratoprosthesis retention, and postoperative complications.A total of 29 eyes of 26 patients received a Boston keratoprosthesis type II during the study period. Patients undergoing operation had corneal blindness because of mucous membrane pemphigoid (51.7%), Stevens–Johnson syndrome/toxic epidermal necrolysis (41.4%), or other ocular surface disease (6.9%). Visual acuity after surgery improved to 20/200 or better in 23 eyes (79.3%) and to 20/30 or better in 10 eyes (34.5%). In patients with at least 1 year of follow-up (n = 21), visual acuity of 20/200 or better was maintained in 12 eyes (57.1%). Of 13 eyes followed-up for more than 5 years, 6 eyes (46.2%) had visual acuity of 20/200 or better at the last follow-up examination. Eyes that did not improve to 20/200 or lost vision during the follow-up had end-stage glaucoma, previous retinal detachment, or age-related macular degeneration. Of the total of 29 eyes, 17 devices (58.6%) were retained without extrusion or replacement during a total follow-up time of 107.9 person-years.The Boston keratoprosthesis type II is a viable option for corneal blindness from severe autoimmune ocular surface diseases.