Primary sclerosing cholangitis (PSC) is a rare, chronic cholestatic liver disease of uncertain etiology characterized by the destruction of the intrahepatic and extrahepatic bile ducts through inflammation and fibrosis. This development of inflammation and fibrosis leads to biliary complications including cirrhosis and ultimately death. Given the uncertainty surrounding the pathogenesis of the disease, many different medical therapies have been studied in the treatment of PSC. However, there currently are no effective medical therapies known to halt progression of disease. Additionally, patients with PSC often develop symptoms and complications that can be managed endoscopically or surgically. This review primarily focuses on endoscopic and surgical approaches that have been studied in the treatment of patients with PSC.