Pheochromocytoma, although rare, is a potentially life-threatening disease, that if recognized early by the physician, can be managed with minimal morbidity. A high degree of suspicion in those patients with new onset hypertension with sudden worsening or development of diabetes mellitus, or a family history of multiple endocrine neoplasia, the neuroectodermal tumors, or simple pheochromocytoma should prompt biochemical confirmation. Surgical removal is the only definitive therapy. Additionally, lifelong follow-up (yearly, initially) is necessary to detect any signs of benign recurrence or malignancy, since these have been reported to occur as long as 41 years after the initial surgical resection.