This brief review focuses on recent advances in the management of functional pancreatic neuroendocrine neoplasms (F-pNEN). F-pNEN are rare malignancies that may be associated with hormonal hypersecretion, leading to diverse clinical syndromes. In the present manuscript, we sought to comprehensively explore the recent developments in the epidemiology, pathophysiology, and clinical manifestations of these tumors, specifically for insulinomas, gastrinomas, glucagonomas, VIPomas, and other uncommon manifestations. The review delves into an expert multidisciplinary approach to the treatment of F-pNEN, emphasizing the pivotal role and complexity of surgical resection, medical therapies, and emerging targeted interventions. We discuss the use of somatostatin analogues, peptide receptor radionuclide therapy (PRRT), and other pharmacological options in managing hormonal hypersecretion and tumor growth. Additionally, we address the challenges associated with metastatic disease and the potential for combination therapies. Moreover, the manuscript highlights the importance of patient-centered care, ongoing research, and collaborative efforts in optimizing treatment outcomes for individuals with F-pNEN.