Primary biliary cholangitis (PBC) is a rare autoimmune liver disease that leads to chronic cholestasis and progressive liver dysfunction. It is often accompanied by extrahepatic symptoms such as pruritus and fatigue, which significantly impair the quality of life. Current treatment options include ursodeoxycholic acid, the standard first-line therapy, along with second-line agents like obeticholic acid, and recently approved seladelpar, and elafibranor. These treatments aim to alleviate symptoms, improve liver function, and slow disease progression. This article focuses on recently approved therapies for PBC, discusses the nuances in their use, and explores the investigational pipeline of novel therapies under development.