Seven cases of primary biliary cirrhosis were reported so far in Korea. We experienced two cases of this disease. One, 47-year-old woman who complained pruritus, easy fatigability, xerostomia and xeropthalmia for 5 years, is diagnosed primary biliary cirrhosis associated with Sjogren's syndrome. The other, 44-year-old woman, who has previous history of Bechet's disease, suffered from arthralgia and jaundice. They showed cholestatic type of liver dysfunction, but had no obstructive lesion in biliary trees. We tried to treat with cholestyramine and ursodeoxycholic acid. We reviewed and summerized all the other reported cases in Korea.