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In the last decade, advances in practice have improved clinical care, driven novel therapeutic options and improved risk stratification tools.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>To provide an overview of the disease characteristics of PBC and review a patient\u2010centred management approach for the clinical team caring for those with PBC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We reviewed the current literature and guidelines on PBC with a focus on management and therapies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A confident diagnosis of PBC is usually made based on serum liver tests and immune serology. Management of PBC should focus on three main \u2018process\u2019 pillars: (a) treat and risk\u2010stratify through use of biochemical and prognostic criteria; (b) manage concurrent symptoms and other associated diseases; and (c) stage disease, monitor progression and prevent complications. With ongoing complexities in management, including a newly licensed therapy (obeticholic acid) and alternative non\u2010licensed treatments and ongoing clinical trials, discussion with PBC expert centres is encouraged.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>PBC is a dynamic disease wherein current treatment goals have become appropriately ambitious. 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