1,2,3,4,5 In childhood, Cushing’s disease, a pituitary adenoma producer of ACTH, isthe most common etiology of Cushing Syndrome over the age of 51. ,6,7,8,9 However, ectopic ACTH secretion is very rare (<1‒3%) in children andadolescents6. ,7,8. This evidence concerns the gene POMC and Cushing syndrome due to macronodular adrenal hyperplasia.