The syndrome of ectopic production of ACTH by non-pituitary neoplasm occur with various types of tu#rnors. Pheochromocytoma is a rare, but one of the important causes of this syndrome. Recently we experienced a 36-year-old male patient with ACTH-producing pheochromocytoma. After left adrenalectomy, serum ACTH level retumed to the normal range. The patient became normotensive without antihypertensive agents and hyperglycemia resolved and insulin therapy was discontinued. Secretion of ACTH was confirmed by immunohistochemical stain.