POMC and Cowden syndrome 1: 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).