Endogenous CS is divided into adrenocorticotropic hormone- (ACTH-) dependent and ACTH-independent etiologies, with the former including Cushing's disease (CD) and ectopic ACTH syndrome (EAS) and the latter including adrenocortical adenoma (ACA), adrenocortical carcinoma (ACC), primary bilateral macronodular adrenal hyperplasia (BMAH), and primary pigmented nodular adrenocortical disease (PPNAD) [1]. Here, POMC is linked to Cowden syndrome 1.