It is difficult to determine whether a pheochromocytoma is benign or malignant, even if histopathology is obtained. According to WHO diagnostic criteria: malignantpheochromocytoma is the appearance of chromophobic cells (metastases) in areas where there is no chromophobic tissue, such as bone, lymph nodes, liver, and lung. The WHO tissue classification of adrenal tumors divides pheochromocyte-associated tumors into two categories: adrenal medullary tumors and extra-adrenal paragangliomas: the former includes benign, malignant PHEO and mixed PHEO/PGL; the latter includes extra-adrenal sympathetic and parasympathetic PGL. The incidence of malignant PGL is about 30% to 40%, and malignant PHEO of the adrenal glands is about 10%. Children with multiple and extra-adrenal accounts for 30% to 43%, of which malignant accounts for 26% to 35%. Metastatic sites are mostly seen in lymph nodes, liver, lung, bone and other organs. However, pathologic histologic features alone cannot predict malignancy or metastasis. For local lesions either adrenal or extra-glandular, if there are no distant metastases (chromophobic cells appearing as foci in areas without chromophobic tissue), it is impossible to determine benign or malignant. According to the statistics of malignant pheochromocytoma/paraganglioma cases in our hospital, about 50% of the patients were considered as benign pheochromocytoma/paraganglioma and were treated with surgery, but after several years, distant metastasis or recurrence with metastasis occurred, and the recurrence time was as short as 3 months and as long as 20 years, so pheochromocytoma/paraganglioma needs lifelong follow-up regardless of benign and malignant. CT/MRI (extra-glandular), 131I-MIBG whole body imaging (whole body can be observed), blood and urine catecholamines, and related symptoms caused by excessive secretion of catecholamines. If postoperative symptoms reappear (such as hypertension, panic, sweating, headache, dizziness, constipation, weight loss, etc.), it should be highly suspected that the tumor has recurred and a comprehensive examination should be performed immediately.