Primary biliary cirrhosis (PBC) is a chronic inflammatory disease of unknown cause that is characterized by the destruction of small intrahepatic bile ducts. It is rare disease in Asia, especially in Korea. We report here a case of a 64-year old female with primary biliary cirrhosis and Sjogren s syndrome who has suffered from generalized pruritis and intermittent dry eye since 6 years ago, and sudden hematemesis. She was treated with endoscopic sclerotherapy, cholestyamine and ursodeoxy- cholic acid. We report the second case of primary biliary cirrhosis withe Sjogren's syndrome in Korea.