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Up to 75% of cases are caused by mutations in the <jats:italic>BRAF<\/jats:italic> gene, whereas <jats:italic>KRAS<\/jats:italic> gene mutation has only been reported in &lt;2% of cases. CFC syndrome is characterized by cardiac abnormalities, distinctive craniofacial dysmorphism, and various cutaneous abnormalities. Musculoskeletal and orthopedic manifestations are also prevalent in patients with CFC syndrome, among which the most common are skeletal deformities and joint laxities. Dysplastic bone disorders, on the other hand, have not been reported in CFC syndrome before. We report on a case of symmetrical polyostotic fibrous dysplasia (FD) in a patient with CFC syndrome with the <jats:italic>KRAS<\/jats:italic>(NM_004985.5):c.57G&gt;C; p.Leu19Phe variant. The FDs were incidentally picked up, and patient was conservatively managed and remained asymptomatic on follow\u2010up. The same variant was reported previously in a patient with Oculoectodermal Syndrome (OES), who developed polyostotic non\u2010ossifying fibroma (NOF). This case explores FD as a possible new clinical feature of CFC syndrome, and when linked to the historical case of OES, explores whether the <jats:italic>KRAS<\/jats:italic>(NM_004985.5):c.57G&gt;C; p.Leu19Phe mutation may potentially contribute to the development of dysplastic bone lesions in patients with this particular mutation.<\/jats:p>","DOI":"10.1002\/ajmg.a.62879","type":"journal-article","created":{"date-parts":[[2022,7,8]],"date-time":"2022-07-08T06:59:49Z","timestamp":1657263589000},"page":"2732-2737","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Fibrous dysplasia in cardio\u2010facio\u2010cutaneous syndrome: A case report and review of literature"],"prefix":"10.1002","volume":"188","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8116-4135","authenticated-orcid":false,"given":"Xiaoao","family":"Dong","sequence":"first","affiliation":[{"name":"Department of Paediatrics KK Women's and Children's Hospital  Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicholas C. Y.","family":"Png","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology KK Women's and Children's Hospital  Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marielle V.","family":"Fortier","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology KK Women's and Children's Hospital  Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jiin Ying","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Paediatrics KK Women's and Children's Hospital  Singapore"},{"name":"SingHealth Duke\u2010NUS Genomic Medicine Centre  Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kenneth P. L.","family":"Wong","sequence":"additional","affiliation":[{"name":"Department of Paediatric Orthopaedics KK Women's and Children's Hospital  Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jonathan T. 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