Focal nodular hyperplasia (FNH) is an infrequent benign tumor of the liver that is generally believed to have no potential for malignant transformation; the coexistence of FNH and hepatocellular carcinoma (HCC) has seldom been reported. Here we describe an exceptional case of simultaneous FNH and HCC in the same patient and discuss the clinical and therapeutic management of FNH on the basis of a review of the literature.