ACTH-independent macronodular bilateral adrenal hyperplasia (AIMAH) is a rare cause of endogenous Cushing's syndrome. Here, massive bilateral adrenal enlargement is accompanied by ACTH-independent hypercortisolism. The detection of ectopic hormone receptors which, according to a new concept, control the cortisol production in AIMAH, offers the opportunity of normalizing the hypercortisolism by pharmacologically influencing the receptor or its ligand. We here present the case of a 46 year old male patient. Using clinical and pharmacological tests we found evidence of ectopic receptors in the AIMAH. After suspicion was erroneously raised that a malignant lesion could be inside of the right adrenal mass, the decision was made to resect both adrenals instead of trying to treat the hypercortisolism by pharmacological means. This surgical approach (bilateral adrenalectomy) has been the standard way of treatment for AIMAH until the new concept of the ectopic receptors was developed.