A recent study from our group reported the largest series of patients with PBMAH to date, including a broad range of phenotypes, from bilateral adrenal incidentaloma without any clinical evidence of endogenous cortisol excess to massively enlarged adrenals associated with severe hypercortisolism and showed that, additionally to the bilateral adrenal nodules, ARMC5-mutated patients constantly present with a cortisol dysregulation, at least with a mild autonomous cortisol secretion [20]. This evidence concerns the gene ARMC5 and adrenal gland hyperfunction.