Originally called carcinoid tumours, neuroendocrine tumours (NETs) are a heterogeneous group of malignancies originating from cells of the neural crest and characterized by the over expression of somatostatin (SS) receptors. They have a variable and often long natural history. They commonly arise from the gastrointestinal (GI) tract (58%), or lung (27%), and can be associated with symptoms resulting from the secretion of hormones or vasoactive peptides. Incidence rates are rising and prevalence is much higher resulting from 5 year survivorship of > 60%. In patients with incurable disease, several systemic options have demonstrated activity but until recently, few had been compared in prospective, randomized controlled trials (RCTs). The treatment paradigms for the management of NETs can be considered to have changed with the development of high level evidence to guide medical therapy arising from RCTs, with recent advances in diagnostic imaging including 68Gallium-DOTATATE PET scans and Peptide Radionuclide Receptor Therapy (PRRT), and the recognition of accurate pathology reporting of tumour grade. Patient advocacy groups such as the Unicorn Foundation have led to a need for greater professional collaborations in providing best care to patients and the establishment of clinical practice guidelines. Historically, therapy was based around surgery and symptom control using SS analogues (SSAs), with other therapies offered in selected centres based on experience and research interests eg liver directed therapies, PRRT. Although chemotherapy has been used in the past for pancreatic (p) NETs this is based on evidence from old studies of low quality by modern standards. Evidence from modern RCTs has confirmed the value of long acting SSAs in delaying tumour progression in GI NETs. In progressive advanced pNETs, the tyrosine kinase inhibitors sunitinib and everolimus have independently shown efficacy in RCTs with sunitinib now approved on the PBS. The Australasian GI Trials Group will be commencing a Phase II study (CONTROL NETs) in 2014 to evaluate the relative efficacy of PRRT in patients with midgut and pNETs. Other trials of new agents are ongoing overseas and in Australia. The increasing number of therapy options has created the need for more formal multidisciplinary treatment centres in order to determine the optimal treatment option in a given patient in a given time of their illness. In all, the greatest paradigm shift in NETs has been the evolution of practice based on multidisciplinary care focused on higher levels of evidence from RCTs.