Although coexisting primary biliary cirrhosis (PBC) and celiac sprue have been described, celiac sprue is sufficiently common in western Europe for chance to explain isolated cases. We screened our patients with PBC for celiac sprue using serum immunoglobulin A endomysial antibody (EmA), with confirmation by duodenal biopsy in EmA-positive patients. Of 57 patients, 6 (11%) had EmA. Four agreed to have a biopsy taken, and all had villous atrophy, yielding a minimum prevalence of 1:14 (7%). Apart from anemia in one patient, none of the four had symptoms or routine laboratory abnormalities suggestive of celiac sprue. None had improvement in liver biochemical tests after 12 to 24 months on gluten-free diets despite the disappearance of EmA. Celiac sprue is common among patients with PBC and they should be routinely screened for this condition. Symptoms wrongly attributed to PBC may respond to gluten exclusion, and both conditions are potent risk factors for osteoporosis.