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]. The gene discussed is POMC; the disease is Cowden syndrome 1.