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We present a 56-year-old patient with a pathogenic variant of the <jats:italic>NF1<\/jats:italic> gene, who suffered from NF1 with lung manifestations and recurrent SSP. It is essential to identify the patients having an increased risk of developing SSP as preventive surgery seem to decrease the risk of new events. Pneumothorax can be a clinical manifestation of NF1 but is not yet widely acknowledged as such.<\/jats:p>","DOI":"10.1136\/bcr-2020-238694","type":"journal-article","created":{"date-parts":[[2021,3,18]],"date-time":"2021-03-18T13:10:29Z","timestamp":1616073029000},"page":"e238694","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Spontaneous pneumothorax as a clinical manifestation of neurofibromatosis type 1"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3492-0705","authenticated-orcid":false,"given":"Tine","family":"Lorentzen","sequence":"first","affiliation":[{"name":"Clinical Institute, University of Southern Denmark, Odense, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanne","family":"Madsen","sequence":"additional","affiliation":[{"name":"Odense Research Center for Anaphylaxis, Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark"},{"name":"Department of Respiratory Medicine, Odense University Hospital, Odense, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie Jos\u00e9e Zareh","family":"Lausten-Thomsen","sequence":"additional","affiliation":[{"name":"Radiology, Lillebaelt Hospital, Vejle, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anette","family":"Bygum","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics, Odense University Hospital, Odense, Denmark"},{"name":"Department of Clinical Research, University of Southern Denmark, Odense, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2021,3,18]]},"reference":[{"key":"2025082706505080000_14.3.e238694.1","doi-asserted-by":"crossref","first-page":"17004","DOI":"10.1038\/nrdp.2017.4","article-title":"Neurofibromatosis type 1","volume":"3","author":"Gutmann","year":"2017","journal-title":"Nat Rev Dis Primers"},{"key":"2025082706505080000_14.3.e238694.2","unstructured":"World Health Organization . 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