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While certain findings, including relative or absolute macrocephaly, are typical for most RASopathies, other findings are more common in certain conditions, with rare low\u2010grade gliomas in Noonan syndrome (NS); Chiari 1 malformation and tethered cord in Costello syndrome (CS); and variable structural anomalies including heterotopia and hydrocephalus in cardio\u2010facio\u2010cutaneous syndrome (CFC). We performed a literature review and present aggregate data on the common and uncommon CNS manifestations in individuals with RASopathies. A gene\u2010based approach to defining risk for specific abnormalities may be considered. However, limited information on the CNS findings of rare RASopathies, such as autosomal recessive<jats:italic>LZTR1<\/jats:italic>\u2010related NS or<jats:italic>PPP1CB<\/jats:italic>\u2010related NS with loose anagen hair (NSLH), is currently available. Thus, consideration of the RASopathies as a group of distinct syndromic conditions with shared underlying causes and overlapping clinical presentations remains relevant, and individuals with a RASopathy are at risk for many findings seen in these conditions.<\/jats:p>","DOI":"10.1002\/ajmg.c.32023","type":"journal-article","created":{"date-parts":[[2022,12,1]],"date-time":"2022-12-01T18:51:26Z","timestamp":1669920686000},"page":"494-500","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Central nervous system involvement in individuals with<scp>RASopathies<\/scp>"],"prefix":"10.1002","volume":"190","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3991-135X","authenticated-orcid":false,"given":"K. 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