The differential diagnosis of an abdominal mass in an infant or young child includes hepatoblastoma. This rare embryonal neoplasm of the liver is linked to several congenital abnormalities and may be associated with maternal occupational exposure to metal fumes, petroleum products, or paints. Alpha-fetoprotein is a useful tumor marker that may also be elevated with hepatocellular carcinoma. The relatively better prognosis of hepatoblastoma, if completely resected, mandates its differentiation from hepatoma. The fetal subtype of hepatoblastoma seems to represent a "favorable" histology pattern.