Multiple endocrine neoplasia type 2A (MEN-2A) is an autosomally dominant familial disorder and includes pheochromocytoma (PH), medullary thyroid carcinoma (MTC) and occasionally parathyroid hyperplasia. We present a case of MEN-2A diagnosed as bilateral adrenal PH and MTC. Preoperative prazosin was effective for improving abnormal hemodynamic state. Intraoperative cardiovascular changes were controlled by the use of prostaglandin E1, nicardipine and diltiazem. Following bilateral total adrenalectomy, total thyroidectomy was performed under hormone replacement therapy. By the screening test for the patient's family, his son was diagnosed MEN-2A. He underwent excision of adrenal PH and MTC. In the treatment of MEN-2A, there are two important points. First, removal of PH should be scheduled before removal of MTC. Second, a screening test for the patient's children should be carried out.
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