PBMAH is often diagnosed incidentally in patients with CS presentation.[2] Three primary pathogeneses have been proposed for PBMAH, including aberrant adrenal expression of ectopic receptors,[4] local ACTH production in the adrenal glands,[5] and genetic mutations such as Armadillo Repeat Containing 5 (ARMC5) mutations.[6] Here, we present a rare case of PBMAH, which experienced intermittent fever for 3 years and was diagnosed with mild CS in the process of the workup for fever of unknown origin (FUO). The gene discussed is POMC; the disease is Cowden syndrome 1.