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Delayed recognition of progressive familial intrahepatic cholestasis (PFIC) can contribute to ongoing symptoms, disease progression, and missed opportunities for appropriate management. This downloadable slideset reviews key considerations for diagnosing PFIC, selecting and interpreting genetic testing, and individualizing treatment decisions based on disease characteristics and patient needs. Additional content highlights management of cholestatic pruritus, the evolving role of IBAT inhibitors, and strategies for lifelong monitoring, surveillance, and multidisciplinary care.