The endogenous hypercortisolism can be ACTH-dependent due to a pituitary ACTH hypersecretion defined as Cushing’s disease (CD) or to extrapituitary hyperproduction of ACTH, defined as ectopic CS or ACTH-independent CS due to adrenal cortisol hypersecretion [1]. Here, POMC is linked to Cowden syndrome 1.