Two cases are reported in which the very infrequently reported association was found of liver cell carcinoma and biliary cirrhosis secondary to congenital biliary atresia. A search of the literature revealed 4 previous reports of cases with similar pathology. Our 2 patients were both operated upon within the first few months of life, at which time congenital biliary atresia was documented, and in 1 instance temporarily corrected. They ran a progressive downhill course until they both received replacement livers, one at 4 years of age and the other at 12, at which times hepatoma was found in the excised cirrhotic livers. One patient is in good health 18 months post-transplantation. The other developed metastases but died of gastro-intestinal bleeding and pneumonia 76 days post-transplantation
