Objective：To promote the diagnosis and treatment in children with pheochromocytoma. Methods：Clinical data of six pa－tients with pheochromocytoma during thirteen years were analyzed retrospectively and literatures were reviewed. Results：Four cases had an increase of vanilla mandelic acid（VMA） within 24 h；six cases had an increase of blood catecholamine（CA） and were found positive mass by ultrasound，CT and MRI examination；four cases had adrenal mass；two cases had extra-adrenal mass（bladder）. Surgi－cal excision was performed in all of cases under general anesthesia and pheochromocytoma was confirmed postoperatively by histologic appearance. Patients were followed up for 1-13 years；five cases had tumor-free survival；only one case revealed tumor metastases by referral examination and died two months later due to disagreement of further treatment. Conclusion：The radical treatment of pheo-chromocytoma is surgical excision. It is crucial to perform preoperative preparations including volume dilatation and management of hypertension，to avoid intraoperative extrusion of tumor and intraoperative occurrence of severe hypertension，and to control blood pressure postoperatively. Differentiating benign from malignant pheochromocytoma by histologic appearance is difficult. In case of child with malignant hypertension，arrhythmia，palpitation，visual disturbance or abdominal pain，pheochromocytoma need to be consid－ered.
