Functional neuroendocrine neoplasm, which secrete hormones and lead to diverse clinical symptoms, pose significant challenges in diagnosis and treatment. Early identification and standardized management are crucial for improving patient prognosis. This article summarized three clinically relatively common cases of Functional pancreatic neuroendocrine neoplasm (F-pNENs), focusing on analyzing their diagnostic key points and standardized therapeutic pathways. It aimed to provide reference for early clinical identification of rare tumors and improvement of standardized multidisciplinary diagnosis and treatment as well as personalized treatment strategies.