Inflammatory pseudotumours (IPTs) of the biliary tract are extremely rare and heterogeneous by aetiology and clinical presentation. They might cause biliary obstruction and mimic cholangiocarcinomas and their final diagnosis is usually achieved only after surgical excision. The most characteristic feature of IPT is the presence of chronic inflammatory cell infiltrates with variable degree of proliferating fibrous tissue. IPTs have the potential for recurrence even after resection and if untreated they can grow causing a variety of symptoms due to compression of the surrounding structures and organs. Despite the significant improvement of modern imaging techniques, preoperative distinction between IPTs of the biliary system and malignancies is extremely difficult. Histological diagnosis poses a clinical challenge because sampling is often suboptimal. Although rare, IPTs should be in the differential diagnosis of patients who present with painless jaundice and no other clinical symptoms or signs characteristics of cholangiocarcinomas.