We extended by 10 years, a follow-up study of 279 patients with primary biliary cirrhosis initially evaluated at the Yale Liver Study Unit between 1955 and 1979. Thirty-six patients (13%) were asymptomatic at the time of diagnosis. Accurate follow-up survival data were available for 247 patients (89%), ranging up to 24 years after the original diagnosis. Median predicted survival of patients in this study from the time of diagnosis is twice as long for patients who present without symptoms compared to symptomatic patients (16 vs 7.5 years, p < 0.0001). However, overall survival of those asymptomatic patients is shorter than that predicted for an age- and gender-matched control population (p < 0.0001), a difference that became apparent only after 11 years of follow up. With a median follow up of 12.1 years, 33% of the asymptomatic patients remained free of symptoms of liver disease, However, once symptoms develop, their survival is similar to those presenting with symptoms. Independent predictors of diminished survival include: elevated bilirubin, increasing age, ascites, advanced fibrosis and the degree of portal bile stasis on liver biopsy. It was not possible to predict which asymptomatic patients would remain symptom free.