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Biliary tract cancer in the setting of IgG4 related disease has been previously described; however, this patient course is novel as it encompasses the spectrum of challenges in IgG4-SC management, including diagnostic uncertainty, risk of infection with immunosuppressive agents, and development of malignancy diagnosed shortly following IgG4-SC diagnosis. We review the literature of management, outcomes, and malignancy risk and furthermore, highlight a promising recent therapy in treatment of IgG4 related disease, inebilizumab.<\/jats:p>","DOI":"10.17998\/jlc.2025.12.09","type":"journal-article","created":{"date-parts":[[2025,12,17]],"date-time":"2025-12-17T01:52:30Z","timestamp":1765936350000},"page":"169-175","update-policy":"https:\/\/doi.org\/10.17998\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["A rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma"],"prefix":"10.17998","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2107-8304","authenticated-orcid":true,"given":"Gordon","family":"Hong","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-1920-5108","authenticated-orcid":true,"given":"Amber","family":"Hussain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0454-6740","authenticated-orcid":true,"given":"Eduardo Thadeu de 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