Context Studies of mortality in celiac disease have not taken small-intestinal pathology into account. Objective To examine mortality in celiac disease according to small-intestinal histopathology. Design, Setting, and Patients Retrospective cohort study. We collected data from duodenal/jejunal biopsies taken between July 1969 and February 2008 on celiac disease (Marsh stage 3: villous atrophy; n=29 096 individuals) and inflammation (Marsh stage 1-2; n=13 306) from all 28 pathology departments in Sweden. A third cohort consisted of individuals with latent celiac disease from 8 university hospitals (n=3719). Latent celiac disease was defined as positive celiac disease serology in individuals with normal mucosa (Marsh stage 0). Through linkage with the Swedish Total Population Register, we estimated the risk of death through August 31, 2008, compared with age-and sex-matched controls from the general population. Main Outcome Measure All-cause mortality.