Summary 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 <103 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.