A 51-year old female was referred to our hospital for the evaluation of huge hepatic mass on routine abdominal sonogram. She was also presenting uncontrolled hypertension. An enhanced abdominal CT and liver MRI showed a 94mm sized large hepatic mass favoring hepatoma or focal nodular hyperplasia and metastatic lesion in right iliac bone. On liver biopsy, histology and immunohistochemistry revealed an extra-adrenal malignant pheochromocytoma. On chest CT, which was performed to identify another metastatic lesion, we could find two metastatic nodules on left lower lung. In fact, She had a history of an extra-adrenal pheochromocytoma in retroperitoneum, which had been completely removed surgically about 6 years ago. Unfortunately, after liver biopsy, she underwent disastrous post-biopsy bleeding and hypovolemic shock. We performed arterial embolization to control hemorrhage and it succeeded. Finally, she was diagnosed with a malignant extra-adrenal pheochromocytoma which was primarily originated in retroperitoneum and developed multiple metastases after several years. An extra-adrenal malignant pheochromocytoma is an exrtremely rare disease and, to the best of our knowledge, this is the first reported case of extra-adrenal pheochromocytoma with liver, lung and bone metastasis in Korea. This case suggest that when a huge hepatic mass resembling nodular hyperplasia is discovered, an extra-adrenal pheochromocytoma shuld be included in differential diagnosis.