Boston type I keratoprosthesis (KPro) long-term outcomes were compared in patients with and without limbal stem cell deficiency (LSCD) in this retrospective study. In the 11 year period 68 procedures were performed in individuals with LSCD, and 105 procedures in individuals without LSCD. Throughout the five year follow-up more eyes with LSCD achieved corrected distance visual acuity (CDVA) >= 20/200 than the eyes without LSCD. However, patients with LSCD were significantly less likely to have pre-KPro glaucoma and require glaucoma surgery. The retroprosthetic membrane formation was significantly less common in eyes with LSCD than in eyes without LSCD (37% vs. 55% respectively). Persistent corneal epithelial defect formation was significantly more common in eyes with LSCD than in eyes without (50% vs. 28% respectively). The occurrence of other post-op complications (infectious infiltrates, corneal necrosis, retinal detachments, sterile vitritis, endophthalmitis and elevated IOP) did not reach statistical significance between two groups. There was no significant difference in retention rates of KPros between eyes with and without LSCD (94% vs. 86% respectively at last follow-up) and no difference in time to keratoprosthesis removal. The majority of retention failures that occurred in eyes with LSCD occurred in patients with Stevens-Johnson Syndrome (SJS). This study reports that Boston KPro is capable of achieving good long-term outcomes in patients with LSCD with strict postoperative regime.