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This systematic review investigates its clinical relevance, anatomical origins, and diagnostic value, particularly in presurgical evaluation. The sign is most commonly associated with mesial frontal epilepsy, particularly involving the anterior cingulate cortex (ACC), though moderate evidence links it to a broader network including insulo\u2010opercular and temporo\u2010parietal regions. Variability in semiologic descriptions and the absence of standardized definitions present challenges to its consistent recognition. Focal cortical dysplasia (FCD) was identified as the most frequent pathological substrate linked to this sign, particularly in MRI\u2010positive cases, while nearly half of the reviewed cases were MRI\u2010negative emphasizing the importance of localizing signs such as the \u201cchapeau de gendarme\u201d in cases of suspected FCD. FDG\u2010PET consistently showed hypometabolism in relevant regions, supporting its utility in identifying the epileptogenic zone in MRI\u2010negative cases. The propagation of ictal discharges across interconnected brain regions highlights the involvement of specific network dynamics in generating this semiology. The \u201cchapeau de gendarme\u201d sign holds significant value in presurgical evaluations, particularly as part of a multimodal assessment integrating neuroimaging and electrophysiological data. Despite the descriptive nature of included studies and limited photo documentation, the sign remains a valuable diagnostic tool. Further research is needed to standardize reporting and clarify its role in guiding surgical management, especially in MRI\u2010negative focal epilepsy.<\/jats:p>","DOI":"10.1002\/epd2.70048","type":"journal-article","created":{"date-parts":[[2025,5,27]],"date-time":"2025-05-27T10:48:34Z","timestamp":1748342914000},"page":"867-882","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The \u201cchapeau de gendarme\u201d sign in focal epilepsy: A\u00a0systematic review"],"prefix":"10.1002","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-5177-9038","authenticated-orcid":false,"given":"Katharina","family":"Moser","sequence":"first","affiliation":[{"name":"Department of Neuropediatrics University Children's Hospital, University of Zurich  Zurich Switzerland"},{"name":"Swiss Children's Rehab University Children's Hospital Zurich, University of Zurich  Affoltern am Albis 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