It has been claimed that 2.5–5 % of all ectopic ACTH syndromes is caused by a pheochromocytoma [5, 10], while we found ACTH secretion in 10 % from medullary cells in a pheochromocytoma and 10 % from adrenal medullary hyperplasia. This evidence concerns the gene POMC and hereditary pheochromocytoma-paraganglioma.