Advances in the treatment of the respiratory complications of cystic fibrosis, including the availability of lung transplantation have led to a greater awareness of the manifestations of liver disease in up to 40% of patients with Cystic Fibrosis (CF). We report the case of an 18 year old female with CF who presented with advanced hepatocellular carcinoma and no prior clinical evidence of chronic liver disease. Hepatocellular carcinoma is usually the most severe manifestation of advanced cirrhosis although its development in non-cirrhotic cases of chronic liver disease has been reported. With the increasing life expectancy of CF patients it is likely that more unusual hepatic complications of this disease may be identified. Greater awareness may perhaps lead to earlier diagnosis in those at risk.