Neuroendocrine neoplasms (NENs) are rare and highly heterogeneous tumors originating from peptidergic neurons and neuroendocrine cells. They can occur in various sites such as bronchopulmonary and gastroenteropancreatic systems. Pancreatic neuroendocrine neoplasms (pNENs) are the most common in our country, with a significant increase in incidence in recent decades. Here, the authors primarily discusse the latest research status on the classification, diagnosis, and treatment of pNENs, aiming to provide guidance and reference for clinical diagnosis and treatment.