Abstract: Objective To summarize the clinical data of 23 ectopic pheochromocytomas collected in Changhai hospital,and to improve the diagnosis and management of this disease. Methods The clinical data of 23 cases of pathologically proven ectopic pheochromocytomas in our hospital were retrospectively reviewed and systematic followed up. Results Total 23 cases of pathologically proven ectopic pheochromocytomas in our hospital between May,1991 and March,2016,10 female patients and 13 male patients with a median age of 50.4 years,were enrolled into this study.The manifestations of ectopic pheochromocytomas were usually typical.Headache was shown in 7 cases(30.43%),palpitations was shown in 5 cases(21.7%),abdominal pain was shown in 5 cases(21.7%),painless hematuria was shown in 2(8.7%),and sweating was shown in 1 cases(4.3%),and other 10 cases of patients without any symptoms(occult occurrence).All of tumors were located in retroperitoneum.The anatomic locations of the ectopic pheochromocytomas were adjacent to the abdominal aorta and the inferior vena cava in 14 cases(60.9%),adjacent to the renal hilum in 4 cases(17.3%),and adjacent to the head of pancreas in 3 cases(13.0%).Other 2 cases were located in urinary bladder.CgA immunohistochemisty was a significant criterion in pathological diagnosis in our cases.All of the 23 of patients conducted surgical excision.Twenty(87.0%) of them were open surgeries,one cases conducted laparoscopic surgeries,and 2 cases conducted transurethral resection of bladder tumor.Most of the benign patients remained free from recurrence with good prognosis,while 2 malignant cases presented with poor prognosis. Conclusion Ectopic pheochromocytoma is a kind of rare tumors,usually located in retroperitoneum,and adjacent to the abdominal aorta and the inferior vena cava.Most cases have good prognosis,and surgical excision is the mainly treatment.