Cushing syndrome (CS), secondary to ectopic adrenocorticotropic hormone secretion (CS-EAS), was reported initially in 1928, while the link between CS and a nonpituitary tumor as a source of EAS was established in 1962 [1], was recently estimated to constitute up to 18 % of all causes of CS [2–8]. The gene discussed is POMC; the disease is neoplasm.