Neuroendocrine neoplasms (NENs) are derived from neuroendocrine cell system and can have benign or malignant characteristics. They are rare tumors, but have been increasing in incidence over the past 40 years. Patients with NENs may develop symptoms due to primary tumor invasion, metastasis, or from secretion of hormonally active tumor substances. Multiple imaging modalities are used for diagnosis and staging, including specialty scans such as 111In pentetreotide (Octreoscan) and 68Gallium-DOTATATE, along with endoscopy, endoscopic ultrasound, and biochemical marker testing. Treatment involves both surgical approach, for both primary and metastatic lesions, as well as medical management for symptom management and disease progression. This article will review the current clinical knowledge regarding the diagnosis, treatment, and prognosis of these fascinating neoplasms and the associated hormonal syndromes.