Abstract: Primary biliary cholangitis (PBC) is characterized by granulomatous destruction of the interlobular bile duct, causing intrahepatic cholestasis, and eventually leading to liver fibrosis, portal hypertension, liver cirrhosis, liver failure and death. Pruritus is a prominent extrahepatic symptom in patients with PBC, and more than two-thirds of patients will develop itching in the course of the disease, seriously affecting the quality of life of patients. The molecular mechanism of PBC-related pruritus is still a hot topic at present, involving itching-causing bile acid, lysophosphatidic acid, endogenous opioid peptide, 5-hydroxytryptamine and steroid metabolites. This article reviewed the current researches on the pathogenesis and therapeutic targets of PBC-related pruritus, and discussed the evidence-based treatment recommendations and experimental treatment interventions for patients with PBC-related pruritus, so as to provide theoretical basis for the treatment of PBC-related pruritus