Pheochromocytomas are rare, catecholamine-secreting tumors arising most frequently in the chromaffin cells of the adrenal glands. Recent studies have suggested that genetic mutations are more frequent than previously appreciated in patients with these lesions. Advances continue to be made not only in the genetic evaluation of these patients but also in the biochemical confirmation and tumor localization. Surgery remains the definitive treatment, and advances in laparoscopic techniques as well as cortical-sparing procedures have reduced the morbidity associated with tumor resection.