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The dysgnathia and oro\u2010pharyngo\u2010laryngeal abnormalities in HFS are virtually identical to those found in the agnathia\u2010otocephaly complex (AOC), of which severe forms can include holoprosencephaly (HPE), synotia\u2010melotia, and <jats:italic>situs<\/jats:italic> abnormalities, but not craniosynostosis. We report an infant with HFS who also presented with <jats:italic>situs inversus totalis<\/jats:italic>, which supports its pathogenic overlap with the AOC. The infant showed severe craniofacial asymmetry due to a complex craniosynostosis, microstomia, a hypoplastic tongue, and severe micrognathia, as well as <jats:italic>situs inversus totalis<\/jats:italic>. Exome sequencing did not identify any potentially causal variants. It has been noted that dysgnathia is most commonly associated with four distinctive anomalies: synotia\u2010melotia, HPE, <jats:italic>situs<\/jats:italic> abnormalities, or craniosynostosis. Through a four\u2010way Venn diagram, we identified eight predictable subsets of patients with dysgnathia that cover all possible phenotypic overlaps that may occur among patients with AOC. Using this approach, we identified that HFS should be conceptualized as a form of dysgnathia (or AOC) that is distinguishable by a distinctive presence of craniosynostosis and an absence of anomalies in the brain and ears, along with the <jats:italic>situs<\/jats:italic> abnormalities observed in this case.<\/jats:p>","DOI":"10.1002\/ajmg.a.64045","type":"journal-article","created":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T11:15:16Z","timestamp":1741864516000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["<i>Situs Inversus<\/i> in an Infant With Hypomandibular Faciocranial Syndrome: Clinical Overlap With the Agnathia\u2010Otocephaly Complex"],"prefix":"10.1002","volume":"197","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4626-148X","authenticated-orcid":false,"given":"Jorge Rom\u00e1n","family":"Corona\u2010Rivera","sequence":"first","affiliation":[{"name":"Service of Genetics and Cytogenetic Unit \u201cDr. Juan I. 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