POMC and adrenal gland hyperfunction: She died 42 days after the initial diagnosis.<h4>Clinical discussion</h4>We here present a patient with ACTH-dependent hypercortisolism attributable to a pancreatic NEN who died of progressive cancer after a delay in definitive diagnosis.<h4>Conclusion</h4>Detailed investigation, including systematic endocrine examination and functional imaging studies, are important for precise diagnosis of, and appropriate treatment for, NENs.