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MEN IIa is the rare disorder consisted of thyroid medullary carcinoma, pheochromocytoma, and hyperparathyroidism. We experienced the case in which 42 year-old male patient with thyroid medullary carcinoma and pheochromocytoma complicated by acute myocardial infarction. During the process of conventional treatment of acute myocardial infarction, paroxysmal hypertension occurred for several times. We sought for the cause of paroxysmal hypertension, and found pheochromocytoma by the radiologic imaging study and the biochemical study and we found the 4X4 cm sized neck mass by palpation. After stabilizing his blood pressure by the use of phenoxybenzamine, we removed the pheochromocytoma in right adrenal gland and the medullary thyroid cancer, by right adrenalectomy and total thyroidectomy respectively. Thereafter, his subjective symptoms and objective signs were improved. We report the case with review of literatures.