Pheochromocytoma is a rare but important tumor in children. Appropriate evaluation and management are essential for a favorable outcome. Pheochromocytomas are more often multifocal and extra-adrenal in children when compared with adults. This pattern makes the diagnosis, localization, and surgical management more challenging. Nonetheless, the standard biochemical evaluation is usually diagnostic, and MR imaging is currently the best tool for tumor localization. The possibility of a familial syndrome must always be considered and should always be thoroughly evaluated when children present with a pheochromocytoma. Conversely, children with a familial syndrome should undergo annual screening for pheochromocytoma from a young age. Given the greater likelihood of a metachronous recurrence in children, consideration should be given to adrenal-sparing surgery in selected patients. When managed appropriately, the outlook for pheochromocytoma in children is excellent. Unfortunately, the uncommon malignant tumors are still difficult to treat. Perhaps the unique genetic characteristics of pheochromocytoma will ultimately be exploited in the management of the aggressive forms of this disease.