Fibrolamellar hepatocellular carcinoma (FHCC), a rare variant of hepatocellular carcinoma, is becoming more prevalent; however, up until 1999, only 18 cases had been reported in Japan. We recently diagnosed a case of FHCC in a 46-year-old Japanese man who had visited four hospitals before being finally admitted to our department. On admission, he was diagnosed as having multiple liver tumors, with lymph node metastasis and peritoneal dissemination. Both hepatitis B antigen and hepatitis C antibody were negative, and the levels of serum alpha-fetoprotein and PIVKA-II were within normal limits. Under a provisional diagnosis of atypical hepatocellular carcinoma (HCC), a right hepatic lobectomy with resection of the metastatic lymph nodes and peritoneal dissemination was performed. The histopathological diagnosis made by our pathologist was atypical HCC. He underwent another operation for a recurrence in the left external iliac lymph node. It has been 29 months since his first surgery in this hospital and he is progressing well, which led us to establish the diagnosis of FHCC. Moreover, his serum carbohydrate antigen 125 levels have been well correlated with this condition.