Focal nodular hyperplasia (FNH) is a relatively rare benign hepatic tumor, usually presenting as a solitary lesion; however, multiple localizations have also been described. The association of FNH with other hepatic lesions, such as adenomas and haemangiomas has been reported by various authors. We herein report a case of a hepatocellular carcinoma arising within a large focal nodular hyperplasia, in a young female patient.