PurposeTo report both the incidence of pediatric keratoconus (PKC) in a population-based cohort and the risk for undergoing corneal surgery over a 20-year period at a single institution. MethodsThe medical records of all patients <19 years of age diagnosed with keratoconus while residing in Olmsted County, Minnesota, from January 1, 1975, through December 31, 2019, were retrospectively reviewed. The records of patients <19 years with keratoconus examined at our institution from January 1, 2001, through December 31, 2020, were also reviewed. ResultsThe incidence of PKC in this population over the 45-year study period was 2.48 cases per 100,000 people per year (95% CI, 1.67-3.29). The mean age at diagnosis was 15.25 years (range, 7-18) years, and 28 (77.8%) were male. During a mean follow-up of 2.8 years (range, 0-17.3 years), 33 of 71 patients managed at our institution (46%) underwent at least one corneal procedure. In this cohort, the Kaplan-Meier risk of requiring a procedure by 10 years following diagnosis was 60%. ConclusionsThe incidence of PKC in Olmsted County, Minnesota, over a 45-year period was 2.48 cases per 100,000 people per year. Nearly half of the patients managed at our institution over the past 20 years required a procedure during follow-up.