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Overlapping manifestations and terminological confusion often lead to misdiagnosis and delayed care. This narrative review searched eight databases from inception to May 5th, 2026, for articles on headache and visual symptoms. Extracted data were synthesized into a structured diagnostic framework. Key benchmarks were clarified across seven categories. Retinal migraine and migraine with aura are distinguished by monocular\/binocular involvement, duration, and stroke risk. Visual snow syndrome is confirmed as a persistent disorder distinct from migraine aura. Life-threatening vascular emergencies\u2014including giant cell arteritis, posterior reversible encephalopathy syndrome, subarachnoid hemorrhage, cerebral venous sinus thrombosis, and arterial dissection\u2014exhibit unique red-flag patterns and imaging features. Intracranial pressure disorders present with pressure-dependent headaches. Optic neuritis subtypes (MS-associated, MOGAD, NMOSD) differ significantly in visual impairment, imaging lesions, treatment response, and prognosis. Occipital lobe epilepsy is differentiated by ultra-short visual hallucinations with ictal activity. Additional ophthalmic, structural, and infectious etiologies further broaden the differential spectrum. This evidence-based framework standardizes clinical differentiation of headache with visual symptoms. Emphasizing key historical features, red-flag signs, advanced imaging biomarkers, and antibody testing enables accurate classification, timely intervention, prevents irreversible vision loss and fatal complications, and optimizes long-term prognosis.<\/jats:p>","DOI":"10.1177\/00368504261477665","type":"journal-article","created":{"date-parts":[[2026,8,12]],"date-time":"2026-08-12T23:36:58Z","timestamp":1786577818000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["When headache meets visual disturbance: An evidence-based review of common and critical entities"],"prefix":"10.1177","volume":"109","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-2524-6605","authenticated-orcid":false,"given":"Shusheng","family":"Jiao","sequence":"first","affiliation":[{"name":"Bethune International Peace Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaocui","family":"Han","sequence":"additional","affiliation":[{"name":"Bethune International Peace Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,8,12]]},"reference":[{"key":"e_1_3_4_2_2","first-page":"e91028","article-title":"Differentiating Visual Symptoms in Retinal Migraine and Migraine With Aura: A Systematic Review of Shared Features, Distinctions, and Clinical Implications","volume":"17","author":"Nordin BA","year":"2025","unstructured":"Nordin BA. 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