A 64-year-old man with HBV-related cirrhosis presented with a liver nodule measuring 2.8 cm revealed by a routine ultrasound and concomitant increased alpha-fetoprotein (AFP) up to 400 UI/l. Contrast-enhanced CT was suggestive of hepatocellular carcinoma (HCC) and the patient underwent laser ablation procedure. Five months later, because of raised AFP up to 1600 UI/l, ultrasonography and abdominal CT were repeated, showing an increased diameter of liver nodule, measuring 3.8 cm. The patient underwent down-staged trans-arterial chemoembolization (TACE) and then was entered into the active liver transplant (LT) list. Lamivudine was already started and the patient underwent LT showing HBV-DNA serum levels <10(3) log/copies at the time of surgery. Pathological analysis performed on the explanted liver showed, instead of the suspected HCC, hepatic yolk sac tumor with the presence of typical 'Schiller-Duval bodies'. The first 12 months of postoperative follow-up were excellent, with no evidence of tumor recurrence.