An asymptomatic 38-year-old woman was admitted to the hospital because of liver tumors which were found at a medical check-up. On admission she had mild anemia. Physical examination indicated a hen egg sized abdominal mass in the left upper quadrant of the abdomen. Computed tomography showed three liver tumors and a soft tissue density tumor in the left upper abdominal cavity. A biopsied specimen of the liver tumors demonstrated hepatocellular carcinoma. Right hepatic lobectomy with total caudate lobectomy and partial resection of the left hepatic lobe where a tumor was found and partial resection of the small intestine including the mesenteric tumor were preformed. A pathological study revealed paraganglioma with liver and lymph node metastases. Postoperatively, examination of a preoperatively stored serum sample showed that serum level of catecholamines such as adranaline and noradranaline were within normal limits, whereas the serum dopamine level was elevated to 850pg/ml. The patient is well and has been free from recurrence two years after the surgery.