Cushing’s syndrome caused by an ectopic secreting of adrenocorticotropic hormone (ACTH) was not common. Furthermore, the ACTH-secreting pancreatic neoplasm was extremely rarer. We present a 27-year old female patient suffering from a pancreatic neuroendocrine tumour (p-NET) with extensive pelvicmetastases, which could secrete ACTH and cause Cushing’s syndrome. The postoperative pathologic examinations of this patient prompted pancreatic poorly differentiated neuroendocrine tumour with extensive metastases of bilateral ovarian, uterus and pelvic peritoneum. The immunohistochemical staining of her tumour tissues was positive for Chromogranin A, Synaptophysin and ACTH. The main aim of this article is to share the experience of her diagnosis and treatment and to review the relevant literatures, with an emphasis on discussing the possible transfer modes. Moreover, we strongly suggested that a careful examination of the pelvic cavity during the follow-up of patients who was diagnosed as ACTH-secreting p-NET should also be carried out.