The sympathetic and parasympathetic paraganglia are the site of pheochromocytomas and paragangliomas. The adrenal pheochromocytoma and the abdominal paraganglioma derive from the sympathetic nervous system. The parasympathetic paragangliomas are predominantly localized in the head and neck region. Most pheochromocytomas are hormonally active and benign. The abdominal paragangliomas usually correspond to adrenal pheochromocytomas, but are more commonly malignant. The parasympathetic paragangliomas are usually benign, hormonally inactive and are most commonly located in the bifurcation of the carotid artery.