Primary sclerosing cholangitis (PSC) is a rare disease, autoimmune in nature, characterized by biliary strictures, chronic cholestasis, and progressive liver dysfunction. While its pathophysiology differs from primary biliary cholangitis (PBC), therapeutic targets still focus on bile acid regulation. PSC currently has no approved therapies, although several novel agents are under investigation. Cholestatic pruritus, a significant symptom in PSC and PBC, is now recognized as an approvable indication, with emerging therapies showing promise. This article highlights the investigational pipeline for PSC and cholestatic itch.