Hepatocellular carcinoma in the native liver of a 38-year-old female patient with biliary atresia

•A 38-year-old female with biliary atresia suffered from HCC in her native liver.•HCC was resected but recurred soon and the patient died in spite of chemotherapy.•Cholangitis and/or child delivery might be risk factors for HCC in elderly biliary atresia patients.

We report a rare case of hepatocellular carcinoma in native liver in a case of biliary atresia. The patient was a 38-year-old female with three children who had an aggressive tumor, resulting in her subsequent death. We also review 14 reports, published previously in the English language medical literature, concerning hepatocellular carcinoma originating from native liver in biliary atresia cases and discuss the possible etiology, and propose more careful follow up for the patients with biliary atresia who suffer from repetitive cholangitis and/or experience the child delivery.