The authors expose the epidemiological features of PBC and outline the role of the immunologic disorders in the disease's pathogenesis. The main clinic and laboratory features of the PBC, its course and pathology are also synthesized. The authors describe the drugs employed in the therapy of the PBC: cortisone, azathioprine, metotrexate, chlorambucil, colchicine, D-penicillamine and hydrophilic bile salts; the favourable risk-benefice rate of colchicine, azathioprine and hydrophilic bile salts is outlined, even if they are poorly active on the disease's course. The use of lyposoluble vitamins is stressed, to improve malabsorption's syndromes. At the end the role of orthotopic liver transplant is regarded.