Primary cholestatic liver diseases represent rare diseases which affect liver cells (hepatocytes), outside liver (extra-hepatic) or inside liver (intra-hepatic) bile ducts and lead to disturbance in production or flow of bile. Primary biliary cholangitis or primary biliary cirrhosis (PBC), how the disease was called until recently, is one of the causes of liver cholestasis. Progressive damage to small bile ducts inside the liver can lead to cirrhosis and liver function failure. Primary sclerosing cholangitis (PSC) can cause both intra-hepatic and extra-hepatic damage, depending on which bile ducts inflammatory process affects. It is characterized by chronic inflammation followed by fibrosis of the bile ducts with consequent segmental obliteration of the lumina. In both diseases, patients may present with itch and skin jaundice, and out of laboratory findings with elevated alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT). In PBC, anti-mitochondrial antibodies are also elevated, while the PSC is determined by magnetic resonance cholangiopancreatography. Diseases can be complicated by development of cholestasis, inflammatory (autoimmune) events, liver cirrhosis or tumor development. Due to its ability to slow down the course of the disease, ursodeoxycholic acid is therapy of choice for PBC, while obeticholic acid is the second line treatment. There is no proven effective drug therapy for the PSC, but endoscopic and operative treatment are used. Complications of long-term cholestasis in the form of malabsorption and itching require symptomatic treatment, although itching is sometimes difficult to treat successfully. Liver transplantation is the method of choice in the final stage of both diseases.