To describe the incidence and distribution of eye diseases affecting children in the first year of life in Olmsted County, Minnesota. We conducted a population-based, retrospective medical record review of infants (≤1 year of age) residing in Olmsted County diagnosed with an ocular disorder from January 1, 2005, through December 31,2014. A total of 4,223 infants were diagnosed with an ocular disorder, yielding an incidence of 20,242/100,000 births per year, or 1 in 4.9 live births (95% CI, 19,632-20,853). The median age at diagnosis was 3 months, and 2,179 (51.5%) were female. The most common diagnoses included conjunctivitis, in 2,175 (51.5%), nasolacrimal duct obstruction, in 1,432 (33.6%), and pseudostrabismus, in 173 (4.1%). Visual acuity was decreased in one or both eyes in 23 (0.5%) infants because of strabismus in 10 (43.5%) and cerebral visual impairment in 3 (13.0%). A majority of the infants (3,674 [86.9%]) were diagnosed and managed by a primary care provider, and 549 (13.0%) were evaluated and/or managed by an eye care provider. Although ocular disorders occurred in 1 in 5 infants in this cohort, most conditions were evaluated and managed by primary care providers. Understanding the incidence and distribution of ocular diseases among infants is useful for planning clinical resources.