Gastrointestinal neuroendocrine tumors (GI-NETs) are rare solid tumors that arise from the secretory cells of the neuroendocrine system. The annual incidence of GI-NETs is 3.56 per 100,000, and the incidence has increased over the past 4 decades (Dasari A et al. JAMA Oncol 3(10):1335–1342, 2017). Most GI-NETs are indolent but there is a subset that can be aggressive and metastasize. GI-NETs can be divided into functioning and non-functioning tumors. There is a variety of GI-NETs that have their own distinct entity with different signs and symptoms as well as treatment. Surgery is the mainstay of treatment with best associated long-term survival, but unfortunately many patients present with metastatic disease.