Neuroendocrine tumors are increasingly diagnosed, either incidentally as part of screening processes, or for symptoms, which have commonly been mistaken for other disorders initially. The diagnostic workup to characterize tumor behaviour and prognosis focuses on histologic, anatomic, and functional imaging assessments. Several therapeutic options exist for patients ranging from curative and debulking surgery through to liver-directed therapies and systemic treatments. Multimodal therapies are often required over the patient’s disease history. The management paradigm can be complex but should be focused on curative resections and then on controlling symptoms and limiting disease progression. There are several new systemic therapies that have completed phase 3 studies with new compounds being studied in phase 2. Genetic and epigenetic markers may lead to a new era of personalised therapy in the future.