BADx is recommended as a second-line therapy for occult or metastatic ectopic adrenocorticotropic hormone (ACTH) secretion, as a life-preserving emergency treatment or ultima ratio in patients with severe treatment-refractory ACTH-dependent Cushing’s syndrome (CS) and in the rare event of endogenous adrenal CS due to bilateral disease (3–5). Here, POMC is linked to Cowden syndrome 1.