This represents an extremely rare presentation of PHNET associated with ectopic adrenocorticotropic hormone (ACTH) production and hypercortisolism. As such, it is a unique presentation of an otherwise rare pathology and hence we believe it contributes to the literature on PHNETs by supplementing it with information on an uncommon variation of an infrequent pathology. This evidence concerns the gene POMC and adrenal gland hyperfunction.