Except for a few cases of adrenocortical carcinoma or unilateral adenoma (5), almost all the cases of adrenocorticotropic hormone (ACTH)-independent CS in infancy have been reported to be due to bilateral macronodular adrenocortical disease encountered in cases of McCune Albright syndrome (MAS) (6,7,8,9,10). This evidence concerns the gene POMC and adrenal cortex carcinoma.