Ectopic ACTH pituitary adenomas (EAPA), located outside the sella turcica and on the embryological migration path of Rathke’s pouch (eg nasopharynx, clivus, sphenoidal sinus, cavernous sinus, suprasellar region) are a very rare cause of Cushing’s syndrome (CS) [1]. This evidence concerns the gene POMC and Cushing syndrome due to macronodular adrenal hyperplasia.