Primary biliary cirrhosis (PBC) is believed to be rare in India. We analyzed our data pertaining to patients with PBC seen in a tertiary referral center over a 5-year period. The diagnosis of PBC was based on liver biochemistry, histology and antimitochondrial antibodies, in the absence of biliary obstruction. Five patients, all women, were diagnosed to have PBC. Pruritus, jaundice and fatigue were the most common initial symptoms. Hepatomegaly was seen in 4 of 5 patients. Associated autoimmune diseases were present in 2 patients. All patients presented with mild hyperbilirubinemia (< or = 6 mg/dL) with disproportionately raised serum alkaline phosphatase level. AMA was positive in 4 patients. Liver biopsy showed stage III-IV disease in 3 of 4 patients. The clinical presentation and course of PBC in India are similar to the experience in the West.