Pancreatic neuroendocrine neoplasms (pNENs) are heterogeneous malignancies originating from pancreatic endocrine cells, constituting 2-5% of all pancreatic tumors with rising incidence. They are classified into well-differentiated neuroendocrine tumors (pNETs) and poorly differentiated neuroendocrine carcinomas (pNECs), exhibiting distinct biological behaviors and prognoses. Functionally active tumors manifest hormone-related syndromes (e.g., insulinoma-induced hypoglycemia, gastrinoma-associated ulcers), while non-functional pNENs often present with nonspecific symptoms like abdominal pain or are detected incidentally. Diagnosis relies on multimodal approaches: contrast-enhanced CT/MRI identifies hypervascular lesions; somatostatin receptor imaging (e.g., Ga-68 DOTATATE PET) aids localization; and chromogranin A serves as a key serum biomarker. The definitive and accurate diagnosis of tumors relies on pathological examination. Surgical resection is curative for localized disease, achieving long-term survival in early-stage pNETs. Prognosis varies significantly by grade (G1, G2, G3 or pNEC). Multidisciplinary care is essential for optimizing outcomes. neuroendocrine neoplasms (NENs) are prone to misdiagnosis as other tumors on imaging, necessitating heightened clinical awareness. pNENs may exhibit atypical imaging presentations, thus are prone to misdiagnosis, necessitating heightened clinical vigilance.