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Here we report the first case of this rare malignancy in a young female with situs inversus totalis. Our patient initially presented with early satiety and worsening left-sided abdominal pain. Laboratory values, imaging and biopsies diagnosed a neuroendocrine tumour of primary hepatic origin. However, tumour staging with DOTATATE-PET and FDG PET\/CT suggested incongruency with the biopsied diagnosis and pathology after upfront surgical resection revealed the cholangioblastic variant of intrahepatic cholangiocarcinoma.<\/jats:p>","DOI":"10.1136\/bcr-2025-266833","type":"journal-article","created":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T10:55:19Z","timestamp":1787914519000},"page":"e266833","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Cholangioblastic variant of intrahepatic cholangiocarcinoma in a young adult with situs inversus totalis"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6197-316X","authenticated-orcid":false,"given":"Julie","family":"Thomann","sequence":"first","affiliation":[{"name":"University of Massachusetts Chan Medical School, Worcester, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah 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