This is the first case report of fibrolamellar carcinoma of the liver (FCL) in Japan with reference to the relevant literature. A 56 year-old Korean male with positive HBsAg was complaining of generalized weakness. Alpha-fetoprotein level was elevated and a mass lesion in right lobe of the liver was detected. He underwent a wedge resection, of the liver and the follow-up study failed to show recurrence of the tumor in 15 months. The specimen showed a tumor consisting of a firm, grayish-white, circumscribed solitary mass measuring 4 cm in diameter. Microscopically the tumor was characterized by polygonal and eosinophilic tumor cells and abundant fibrous stroma around the tumor cells in lamellar fashion. FCL is a well-defined disease entirely with a distinct histologic pattern and a favorable prognosis.