Pancreatic neuroendocrine tumors are a diverse group of neoplasms with a generally favorable prognosis. Although they exhibit indolent growth, metastases are seen in roughly 60% of patients. Pancreatic neuroendocrine tumors may produce a wide variety of hormones, which are associated with dramatic symptoms, but the majority are nonfunctional. The diagnosis and treatment of these tumors is a multidisciplinary effort, and management guidelines continue to evolve. This review provides a concise summary of the presentation, diagnosis, surgical management, and systemic treatment of pancreatic neuroendocrine tumors.