Hepatocellular carcinoma associated with arteriohepatic dysplasia

This report documents the occurrence of hepatocellular carcinoma in a 36-year-old man with arteriohepatic dysplasia and a noncirrhotic liver. This case represents one of the oldest patients reported to have arteriohepatic dysplasia and the first with the development of a hepatocellular carcinoma, of which I am aware. Although this tumor has been reported with other familial cholestatic syndromes, the absence of an underlying cirrhosis is of particular interest.