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These are disorders characterized by postzygotic somatic mutation in genes involved in RAS\/MAPK signaling pathway. In this report we describe two patients with linear sebaceous nevus syndrome found to have mutations in codon 146 of <jats:italic>KRAS<\/jats:italic> with evidence of mosaicism. This specific mutation has previously been reported in Oculoectodermal Syndrome and Encephalocraniocutaneous Lipomatosis, two other mosaic RASopathies with predominantly cerebrooculocutaneous manifestations. These findings suggest that, while initially classified as different syndromes, these disorders should be evaluated and managed as a spectrum of related disorders.<\/jats:p>","DOI":"10.1002\/ajmg.a.62422","type":"journal-article","created":{"date-parts":[[2021,7,13]],"date-time":"2021-07-13T12:29:30Z","timestamp":1626179370000},"page":"3825-3830","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Somatic <scp><i>KRAS<\/i><\/scp> mutation affecting codon 146 in linear sebaceous nevus syndrome"],"prefix":"10.1002","volume":"185","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2238-1177","authenticated-orcid":false,"given":"Saloni","family":"Kapoor","sequence":"first","affiliation":[{"name":"UPMC Children's Hospital of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah L.","family":"Scanga","sequence":"additional","affiliation":[{"name":"UPMC Children's 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