We report the case of a young man with chronic hepatitis B infection of probable perinatal acquisition who was diagnosed with hepatocellular carcinoma at a very early stage (BCLC 0) with a highly favorable prognosis. However, the tumor progressed rapidly and the patient died within months. Currently, there are few data that could help to identify cases likely to show rapid progression and which could prompt initiation of aggressive therapies that might prevent or control such progression.