The unique features of this unfortunate case of sporadic MTC and ectopic ACTH CS include: 1) exceedingly elevated urinary free cortisol, 2) reversible combined neurogenic and nephrogenic DI, 3) CS remission with sorafenib without measurable reduction in cortisol concentration, 4) abdominal aortic dissection, 5) jejuno-jejunal intussusception, 6) development of autoantibodies against neuronal ganglionic acetylcholine receptor, and 7) development of acute liver failure. This evidence concerns the gene POMC and Cowden syndrome 1.