A 28-year old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH at other hospital. However the operation was delayed due to poor general condition and abnormal liver function. She revealed severe hypercortisolism, hypokalemic metabolic alkalosis, glucose intolerance and showed rapid downhill course. She was transferred to our hospital for operation but septic complications were strarted already. She was not recovered in spite of desperate operation and died just after moribund discharge. ACTH producing pheochromocytoma is uncommon and literature review indicated high mortality rates due to improper preoperative diagnosis. Proper preoperative recognition and early surgical management is mandatory for better results.