ABSTRACT Purpose To determine the prevalence of ophthalmological findings suggesting an ocular cause for headache or occult neurological disease, among children with headache. Methods Retrospective cross-sectional study on children with headache at a tertiary outpatient ophthalmology clinic. All children underwent sensorimotor, anterior segment, and dilated fundoscopic examinations, with or without cycloplegic refraction. Prevalence of one or more new findings of ocular or occult neurological cause of headache, including glaucoma, uveitis, optic nerve elevation, or possible asthenopia from strabismus or refractive issues. Headache characteristics and associated symptoms were evaluated as risk factors for ocular findings. Results Among 1,878 children with headache (mean age 10 yrs, range 2–18), 492 (26.1%, 95% CI 24.3–28.2%) children had one or more new ocular findings that could cause headache or indicate intracranial disease: refractive issues (342, 18.2%), strabismus (83, 4.4%), optic nerve elevation (51, 2.7%; 26 with papilledema, 25 with pseudopapilledema), uveitis (6, 0.3%), and glaucoma (2, 0.1%). Shorter headache duration was associated with ocular findings (p = .047), but headache frequency, photophobia, nausea/vomiting, and visual changes were not. In univariable analysis, visual changes (p ≤ .001), nausea/vomiting (p ≤ .002), and morning headache (p = .02) were associated with optic nerve elevation. Conclusion An ophthalmologic examination including cycloplegic refraction is indicated in children with headache, as one-quarter have a treatable ocular condition, which may be related to the headache, or sign of intracranial pathology. While nausea, visual changes, or morning headache should raise concern, coincident visual, ocular, or systemic symptoms are not reliable predictors of discovering ocular pathology in a child with headache.