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It mostly results from a post\u2010zygotic <jats:italic>NRAS<\/jats:italic> mutation of neural\u2010derived crest cells, leading to uncontrolled cell growth. Because of the increased knowledge of the genetics underlying CMNS, targeted therapy becomes a promising treatment option. We present a case of CMNS in a newborn. Physical examination at birth showed a giant congenital melanocytic nevus, extending from the occipital to the lower lumbar region. A magnetic resonance imaging scan revealed multiple cerebral and cerebellar parenchymal lesions. Genetic analysis of the cutaneous lesions showed the presence of an <jats:italic>NRAS<\/jats:italic> Q61R mutation. The patient was treated with dermabrasion to reduce the color intensity of the nevus. However, this was complicated by recurrent wound infections and laborious wound healing. At the age of 1\u2009year, the patient had an age\u2010appropriate psychomotor development, without neurological deficits.<\/jats:p>","DOI":"10.1111\/cup.14097","type":"journal-article","created":{"date-parts":[[2021,7,13]],"date-time":"2021-07-13T18:20:19Z","timestamp":1626200419000},"page":"1497-1503","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["An update on congenital melanocytic nevus syndrome: A case report and literature review"],"prefix":"10.1111","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7413-9238","authenticated-orcid":false,"given":"Lilaf","family":"Abdulmajid","sequence":"first","affiliation":[{"name":"Faculty of Medicine and Health Sciences University Antwerp  Wilrijk Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca Maria","family":"Bosisio","sequence":"additional","affiliation":[{"name":"Department of Pathology University Hospitals Leuven, KU 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