Primary biliary cirrhosis is an uncommon type of biliary obstruction that should be briefly mentioned. It occurs predominantly in young or middle-aged women and typically is a slow process. On liver biopsy there is inflammation and destruction of small bile ducts within liver portal areas. Clinically there is pruritus with or without mild jaundice. Steatorrhea is sometimes present. Biochemically, there usually is an ALP and GGT increase more than 3 times the normal limit, increased serum cholesterol level, and normal or mildly increased AST levels. Serum antimitochondrial antibodies are elevated in 90% or more patients. Diagnosis is made on the basis of the clinical and biochemical pattern plus the presence of antimitochondrial antibodies. Liver biopsy may be necessary to differentiate the disease from chronic hepatitis, liver tumor, drug-induced cholestasis, or some other conditions associated with cholangitis.