Thirty-six patients presenting with asymptomatic primary biliary cirrhosis have been followed for a median period of 11.4 yr, extending by 5 yr a previously reported median follow-up study of 6 yr. Life table survival analysis indicates that the overall survival of this subgroup of patients with primary biliary cirrhosis continues to remain similar to that of the general population (p = 0.91). Over this period, 15 patients developed symptoms and 8 patients died, 6 from liver disease; 21 patients remained in an asymptomatic state. Portal granulomas on initial liver biopsy were the only finding that correlated with a normal survival and a continued asymptomatic state (p = 0.03). In contrast, associated autoimmune disorders (thyroiditis, sicca syndrome, CRST syndrome, Raynaud's phenomenon) correlated with decreased survival (p = 0.01). No other clinical, laboratory, or histologic features correlated with survival or the development of symptoms. This extended follow-up study (median 11.4 yr) indicates that many patients with asymptomatic primary biliary cirrhosis have a benign outcome. Although 42% developed signs or symptoms of progressive disease at variable times up to 14 yr from presentation, the group survival remained similar to the general population.