This case describes the clinical course and treatment of a 17-year-old male 
patient with advanced hepatocellular carcinoma (HCC) arising in a non-cirrhotic 
liver. The disease was thought to be caused by a congenital cholestatic syndrome 
associated with intermittent oedema in childhood, resembling the rare Aagenaes 
syndrome. Treatment choices in advanced HCC arising in adolescence are 
discussed.