The sclerosing hepatocellular carcinoma is an unusual subtype of hepatocellular carcinoma characterized by an intense fibrous stroma in which the tubular structures are embedded. Histological diagnosis is difficult, and misinterpretation as cholangiocarcinoma or metastatic adenocarcinomas may occur. It usually presents on non-cirrhotic livers. Metastases located outside of the liver are frequent as well as hypercalcemia and low levels of serum phosphate. We report a case of this rare histopathologic subtype of hepatocellular carcinoma in a 35-year-old woman, asymptomatic and with no previous history of chronic liver disease. It was detected by a routine gynaecological ultrasonography. An important increase of serum alpha-fetoprotein was noted. Extension studies showed bilateral lung metastases. Several palliative therapies were tried out, including epirrubicin, with partial response. Twenty months after the diagnosis the patient's situation is nearly terminal.