We describe a fibrolamellar carcinoma of the liver in a 12-year-old girl and review the literature concerning the occurrence of this distinct hepatic neoplasm in children up to the age of 16 years. The results of our electron microscopic and immunohistologic studies suggest that some cells of this tumor exhibit neuroendocrine differentiation. This finding has important theoretical implications relative to the tumor's histogenesis and practical implications with respect to the search for tumor markers of clinical usefulness and possibly for therapy.