A population-based study of all patients with primary biliary cirrhosis (PBC) diagnosed from 2000–2008 within a geographically defined area of the Netherlands has found that the incidence and prevalence rates of PBC increased over time (P <0.001). 992 patients with PBC were identified resulting in a mean incidence of 1.1 per 100,000 individuals; prevalence of PBC on 1 January 2008 was 13.2 per 100,000 individuals. PBC was not associated with smoking, age of menarche, age at first pregnancy or number of pregnancies.