Extra-adrenal pheochromocytomas, or gangliomas, develop in the paraganglion chromaffin cells of the sympathetic nervous system. They account for 10% of all pheochromocytomas in adults and 30-40% in children. These tumors are usually larger than their adrenal counterpart. The most common site of extra-adrenal phenochromocytomas is the para-adrenal area, but they also occur at the aortic bifurcation, chest, inferior mesenteric and iliac arteries, bladder, heart and brain. In this report, we describe a 13-year-old girl with a pheochromocytoma along the course of the left ureter, a finding which to our knowledge was previously described only once. Clinical presentation, diagnosis, and treatment are similar to adrenal tumors. Patients should be closely monitored with serum and urine catecholamine determination and MIBG scan to detect any recurrence or distant metastasis. Prognosis is usually good if the tumor is detected early to avoid major complications related to catecholamine excess.