Three patients presented with Cushing syndrome characterized by markedly elevated corticotropin (ACTH) levels at the time of diagnosis of the liver lesions (78,336, 33,000, 1056; normal range: 10–50 pg/ml) and had prior histories of ACTH-secreting pituitary tumors (19, 72, and 52 months prior to developing liver metastases, respectively). The gene discussed is POMC; the disease is Cushing syndrome due to macronodular adrenal hyperplasia.