Degenerative changes of intrahepatic bile ducts with lymphocytic infiltration into portal areas have been recognized as the characteristic histopathologic features in the liver with biliary atresia (BA). Occasionally, lymphocytic infiltration into biliary ductal epithelium was noted and has been thought to be mostly the result of nonspecific chronic inflammation. However, this histological presentation looks quite similar to that of acute graft-versus-host disease (GVHD) after bone marrow transplantation (BMT) or acute cellular rejection (ACR) after orthotopic liver transplantation (OLTx). According to this histological similarity, we presumed that the degeneration of intrahepatic bile ducts (DIBD) might have developed with lymphocytic infiltration into biliary epithelial cells (LIBE) as some part of an immunologic reaction, and focused on the DIBD with LIBE in the patients with BA. Liver specimens obtained from 31 patients with BA at the time of primary Kasai operation and 3 patients at the time of reoperation were reviewed histologically, and investigated DIBD with LIBE. Nine patients with choledochal cyst (CBD) and 5 patients with neonatal hepatitis (NH) were added to this study as a control population. All patients with BA presented with DIBD with LIBE. On the other hand, only 2 patients with CBD presented with DIBD with LIBE, and no patients with NH presented with DIBD with LIBE. The DIBD with LIBE is not limited to patients with BA, but it seems to be much more serious in this group. These findings may suggest that some sort of immunologic factors contribute to the persistent DIBD in the patients with BA.(ABSTRACT TRUNCATED AT 250 WORDS)