ACTH-independent CS is primarily caused by unilateral adrenal adenomas (70%) and adrenal carcinomas (20–30%).[10,11] Rare causes include bilateral macronodular adrenocortical disease, bilateral micronodular adrenal hyperplasia (isolated or part of the Carney complex), McCune–Albright syndrome, and bilateral adrenal adenomas or carcinomas.[12–14]. This evidence concerns the gene POMC and adrenal cortex adenoma.