We present three siblings (out of four) with intrahepatic cholestatic disease and cirrhosis. Two of the siblings, a 33-year-old woman and a 34-year-old man had advanced liver disease- with the liver histology showing established cirrhosis with chronic cholestasis and excess copper accumulation. Both died two years later due to hepatic encephalopathy. The third sibling, a 37-year-old man on routine check-up was found to have abnormal liver functions. The liver biopsy showed marked bile ductular proliferation with bridging fibrosis, reduction in interlobular bile ducts, and excess copper accumulation. The presence of antimitochondrial antibody in the serum in 1 in 320 dilutions in all three patients and 1 in 80 dilutions in the oldest healthy sibling and hypergammaglobulinemia in all the siblings confirmed the diagnosis of familial primary biliary cirrhosis. Antinuclear and smooth muscle antibodies were not present. Clinical and biochemical improvement has been noted in the third sibling after therapy with ursodeoxycholic acid.