Several compounds have recently been approved for the systemic treatment of advanced well-differentiated neuroendocrine tumours (NET) of gastroenteropancreatic (GEP) or lung origin. Based on the PROMID and CLARINET trials, somatostatin analogues (SSA) are the preferred first-line approach for all GEP-NET and offer—in addition to antiproliferative effects—durable symptomatic relief for hormonally active tumours. The mTOR inhibitor everolimus has been approved for progressive GEP- and lung-NET and is a widely used drug in this setting. Furthermore, recent results have underlined the high efficacy of somatostatin-receptor targeting radionuclide therapy (PRRT) in somatostatin-receptor positive midgut tumours and PRRT is now considered standard treatment for midgut-NET progressing on SSA. The optimal application of PRRT in somatostatin receptor positive NET with non-midgut site is currently an issue of discussion and should be decided on an individually basis in multidisciplinary boards. Following new insights in the genetic landscape of NET, “hot topics” in recent months include optimal treatment of the recently defined NET G3 and preliminary data on immunotherapy.