The relationship between survival and 25 clinical and histologic variables was studied in 195 patients (171 women, 24 men) who satisfied stringent criteria for the diagnosis of primary biliary cirrhosis. The mean duration of follow-up was 6 yr (range 0-17). One hundred and sixteen patients died, 84% as the result of liver disease and 16% from nonhepatic causes. Using the Kaplan-Meier estimate, we calculated the mortality from liver disease to be 40% after 5 yr and 60% after 10 yr. Ascites, serum bilirubin level, variceal hemorrhage, and age were identified as independent clinical risk factors, and extent of hepatic fibrosis, bilirubinostasis, and Mallory's hyalin were identified as independent histologic risk factors correlating with reduced survival.