This report deals with perioperative treatment for a very rare disease called ectopic ACTH-producing pheochromocytoma in a 55-year-old woman. She showed high norepinephrine, cortisol, and ACTH levels, and diagnosed as ectopic ACTH-producing pheochromocytoma, by MIBG scintigraphy. An alpha-blocker and fluid loading for pheochromocytoma, and an adrenal cortex hormone synthesis inhibitor for Cushing's syndrome, were given simultaneously as a preoperative treatment. Propofol with nitrous oxide-oxygen-sevoflurane and epidural anesthesia were used for excision of the tumor, and the procedure was performed without any marked changes in hemodynamics. Although ectopic ACTH-producing pheochromocytoma is a very rare disease, it can be managed safely by appropriate preoperative treatment for pheochromocytoma and Cushing's syndrome.