Background: The aim of this study was to investigate whether changes in lymph node size in the hepatoduodenal ligament reflect changes in biochemical and histologic markers of cholestasis, hepatocellular damage, and humoral immunoreactivity in patients with primary biliary cirrhosis (PBC). Methods: Twenty-three consecutive patients were examined with repeated liver biopsies, laboratory tests, and ultrasonographic examinations of the hepatoduodenal ligament. The effect of treatment with ursodeoxycholic acid (UDCA) on these factors was also studied. Results: A correlation was found between changes in lymph node size and changes in markers of hepatocellular damage, cholestasis, and humoral immunoreactivity and also with intralobular inflammation. In the UDCA-treated patients the node size decreased slightly during 2 years of treatment, whereas it was unchanged in the control group (p = 0.0528). Conclusions: Changes in the size of the lymph nodes in the hepatoduodenal ligament may reflect inflammation and cholestasis in PBC and thus be a valuable indicator in the followup of patients with this disease.