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. This evidence concerns the gene POMC and pancreatic neuroendocrine neoplasm.