Ectopic syndrome related to an ACTH-secreting tumor represents 12% to 17% of Cushing’s syndrome cases.[8] Ectopic ACTH production is mainly associated with small-cell carcinoma of the lung, but can also be found in various neuroendocrine tumors, such as bronchial, thymic, or pancreatic carcinoid, medullary carcinoma of the thyroid and pheochromocytoma. Here, POMC is linked to Cushing syndrome due to macronodular adrenal hyperplasia.