Fibrolamellar hepatocellular carcinoma (FLH) is a variant of hepatocellular carcinoma (HCC) with distinct clinical, histologic and prognostic features different from conventional HCC. Herein, we present a 14-year-old girl with a palpable mass over the right upper -quadrant of the abdomen. A well-defined mass in the left liver with heterogeneous density and a central linear band was demonstrated by ultrasound and computed tomogram. FLH was proved by the histopathology study of a liver specimen taken from an echo-guided liver core needle biopsy before surgery and from a left hepatectomy. No tumor recurrence was detected by ultrasound in the 2-year follow-up.