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Positron emission tomography CT scan done due to the suspicion of malignancy suggested a large right hydropneumothorax, with diffuse soft tissue thickening and fat stranding. Thoracoscopy-guided pleural biopsy was done, which revealed storiform fibrosis, fragments of fibro-collagenous and fibrofatty tissue with dense lymphoplasmacytic infiltrates, obliterative phlebitis, focal bands of sclerosis and IgG4-positive plasma cells in more than 30% of the plasma cells. A diagnosis of IgG4-related disease was made. The child was treated with steroids and mycophenolate mofetil and the child is doing well.<\/jats:p>","DOI":"10.1136\/bcr-2025-265486","type":"journal-article","created":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T21:30:18Z","timestamp":1751405418000},"page":"e265486","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Paediatric IgG4-related disease manifesting as recurrent pleural effusion"],"prefix":"10.1136","volume":"18","author":[{"given":"Umaserma","family":"Bharathi","sequence":"first","affiliation":[{"name":"Paediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5911-3179","authenticated-orcid":false,"given":"Padmasani","family":"Venkat Ramanan","sequence":"additional","affiliation":[{"name":"Paediatrics, Sri Ramachandra Institute of Higher Education and Research, 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