To the best of our knowledge, this is the first case of ACTH-secreting NET, with improvement of symptoms and signs of hypercortisolism and normalization of the serum ACTH level after bilateral adrenalectomy, with long term of remission, complicated by the recurrence of the tumor in the lung with multiple bone metastasis after 11 years. This evidence concerns the gene POMC and adrenal gland hyperfunction.