Background/Aims: Primary biliary cirrhosis (PBC) is a rare disease in Korea and only 28 cases have been reported so far. We have experienced 11 cases of PBC and analyzed its clinical and histological features. Methods: We reviewed 11 patients with PBC diagnosed by the presence of antimitochondrial antibody and pathologic findings of bile duct injury, retrospectively. Results: The mean age was 47.1±9.5 years (male 35.5±0.5, female 49.7±8.7 years) and the male to female ratio was 1:4.5 (male 2/11, female 9/11). The most common initial presentation was incidentally detected by abnormal results of liver function tests (36.4%). The laboratory findings revealed cholestatic pattern with markedly elevated levels of alkaline phosphatase (996.5±449.0 IU/L) and γ-GTP (385.7±202.3 IU/L) in all patients. Pathologic findings revealed: destruction of the intrahepatic bile ducts in 10 patients (90.9%), proliferation of bile ductules in 9 patients (81.8%), infiltration of inflammatory cellls in portal tracts in 10 patients (90.9%), infiltration of inflammatory cells in hepatic parenchyma in 5 patients (45.5%), septal fibrosis (scarring) in 6 patients (54.5%) and cirrhosis in 4 patients (36.4%). The 7-year-survival probability according to Mayo Clinic prognostic index formula is relatively good (0.59-1.0). Conclusions: The clinical characteristics of PBC are similar to those of western countries. However, the case in which initial presentation was incidentally detected by abnormal results of liver function tests was more common than in western countries.