Abstract: Clinically, extra- adrenal pheochromocytoma is unfrequently- encountered, and malignant pheochromocytoma is rare. It can be found everywhere in the body, so vigilance should be heightened for the tumors occurring at the predilection sites, without a conclusive clinical diagnosis. Localization diagnosis of the tumors resorts to the imageological examinations, such as ultrasonography, CT and MRI, etc. Surgical management is the first choice for management of the malignant extra - adrenal pheochromocytoma. When treating the tumor of this kinds, routine pathological examinations fail to correctly discriminate the tumor stages, and an all- round decision, including the patients' clinical situation, an intra- operative exploration, postoperative pathological results and parameters from other auxiliary diagnosis. Palliative therapy involving cyclophosphamide, vincristine and dacarbazine have been conducted for malignant pheochromocytoma. ¹³¹I- MIBG and 123^I- MIBG may also be used. A postoperative follow- up study is of significance in the malignant pheochromocytomas, as well as in the benign ones.