PURPOSE: To assess outcomes and glaucoma management in eyes with aniridia following Boston type 1 Keratoprosthesis (KPro) implantation. DESIGN: Retrospective, interventional comparative case series. METHODS: POPULATION: Patients with aniridia and patients with other preoperative diagnoses (excluding Stevens-Johnson syndrome, mucous membrane pemphigoid, and congenital disorders) who underwent KPro implantation at Massachusetts Eye and Ear with at least 2 years of follow-up. One eye per patient was selected based on the longer follow-up time. MAIN OUTCOME: Intermediate and long-term outcomes related to glaucoma. RESULTS: The aniridia (n=22) and comparison (n=61) groups had similar preoperative visual acuity (VA, mean ± standard deviation, 1.86±0.52 LogMAR, p=0.33) and follow-up time (65.6±26.3 months, p=0.25). Prior to KPro implantation, eyes with aniridia had more glaucoma (76.2%) and glaucoma surgery (57.1%) than comparison eyes (51.8%, p=0.053; 23.2%, p=0.005, respectively). More Ahmed valves were co-implanted with KPro in aniridia (47.6%) versus comparison eyes (17.9%, p=0.008). At final follow-up, more aniridia eyes had glaucoma (90.5%) than comparison eyes (64.3%, p=0.02), but the two groups had similar percentages of eyes with cup-to-disc ratio (CDR) >0.8 (23.8% vs. 30.4%, p=0.57) or CDR progression of ≥0.2 (42.9% vs. 44.6%, p=0.89, respectively). None of the eyes with prophylactic tube implantation developed glaucoma. Eyes with and without aniridia did not differ in post-KPro VA improvement (72.7%, 72.1%, p=0.96), and final VA (1.28±0.79 LogMAR, 1.23±0.98 LogMAR, p=0.51). CONCLUSION: Despite a higher glaucoma prevalence, eyes with aniridia achieved similar VA as comparison eyes with more than 5 years of mean follow-up time. Boston KPro offers satisfactory visual rehabilitation in aniridia when glaucoma is managed aggressively.