This American Association for the Study of Liver Diseases (AASLD) 2018 Practice Guidance on Primary Biliary Cholangitis (PBC) is an update of the PBC guidelines published in 2009. The 2018 updated guidance on PBC includes updates on etiology and diagnosis, role of imaging, clinical manifestations, and treatment of PBC since 2009. The AASLD 2018 PBC Guidance provides a data-supported approach to screening, diagnosis, and clinical management of patients with PBC. It differs from more recent AASLD practice guidelines, which are supported by systematic reviews and a multidisciplinary panel of experts that rates the quality (level) of the evidence and the strength of each recommendation using the Grading of Recommendations Assessment, Development and Evaluation system. In contrast, this guidance was developed by consensus of an expert panel and provides guidance statements based on formal review and analysis of published literature on the topics. The quality (level) of the evidence and the strength of each guidance statement are not rated.