Primary biliary cirrhosis often presents insidiously but progresses relentlessly in its later stages. Asymptomatic patients, however, may have a relatively benign course. Clinical, laboratory and histologic characteristics support an autoimmune pathogenesis. The complications of primary biliary cirrhosis are secondary to severe cholestasis, portal hypertension and progressive hepatocellular dysfunction. Currently, treatment is only supportive; no drug regimen has been shown to alter the disease course.