Primary sclerosing cholangitis (PSC) is an immune mediated, chronic, cholestatic liver disease causing inflammation and fibrosis of the intrahepatic and extrahepatic biliary tree. PSC is strongly associated with inflammatory bowel disease, especially ulcerative colitis, predominantly affects males and can lead to the development of portal hypertension, cirrhosis and its complications and cholangiocarcinoma. Patients are usually asymptomatic and present with abnormal liver chemistries in a cholestatic pattern with abnormal imaging of the biliary tree. Currently there are no effective medical therapies to prevent disease progression and treatment is primarily for symptomatic relief. Advanced liver disease is treated with liver transplantation. Due to the high risk of the development of cholangiocarcinoma in patients with PSC, screening of the biliary tract and evaluation of suspicious findings should be performed regularly.