In 1902, Nicholls described a tumor originating from a pancreatic islet cell lineage [1]. This was the first report of a pancreatic endocrine tumor (PET). These uncommon tumors occur in approximately 1 out of 100,000 people [2–4]. The incidence in autopsy studies is as high as 1.5% [5]. Pancreatic adenocarcinoma is much more common than its endocrine counterpart with a ratio of pancreatic adenocarcinomas to PETs of approximately 125 to 1 [6]. PETs are commonly associated with a clinical syndrome directly related to a hormone secreted by the tumor. Included among these hormone-secreting tumors are insulinomas, gastrinomas, glucagonomas, and somatostatinomas. Not every pancreatic tumor secretes a hormone resulting in a pathologic syndrome of clinical symptoms. These nonsyndromic tumors are referred to as nonfunctioning PETs and comprise 30 to 40% all patient with PETs [7–10]. Although these tumors typically behave in a less aggressive manner than their pancreatic adenocarcinoma counterpart, PETs metastasize to the liver more commonly than any other neoplasm with the exception of colon cancer [11]. If insulinomas are excluded from the analysis, 50–60% of PETs have metastasized at the time of diagnosis (Table 1). [7, 12, 13] The most common cause of death from PETs is hepatic failure [2].