Pancreatic neuroendocrine tumors (PNETs) are primary neoplasms of the pancreas that arise from endocrine cells. They are very rare, with annual incidence <4–5 per 100,000 individuals. Most cases arise sporadically, while others are associated with clinical syndromes such as multiple endocrine neoplasia type I (MEN1), von Hippel-Lindau (VHL) syndrome, neurofibromatosis type I (NF1), and tuberous sclerosis. Twenty-five percent of all PNETs are functional (i.e., associated with a hormonal syndrome). Surgical resection is the mainstay of treatment and is recommended for (1) functional tumors regardless of size, (2) nonfunctional tumors measuring 2 cm or greater, and (3) selective cases of advanced disease with resectable metastases. PNETs vary greatly in their metastatic potential and a significant proportion of patients present with metastatic disease—most often to the liver and peri-pancreatic lymph nodes. Systemic therapy—including chemotherapy and targeted therapy to the somatostatin receptor—is used in unresectable patients; in contrast, such therapies have no defined role for adjuvant treatment.