Surgery is the only curative treatment for sporadic gastrinomas and has been shown to decrease the rate of development of liver metastases, to increase disease-free survival and to result in a long-term cure in 20–45 % of patients. However, as gastrinomas can arise in different sites, their site of origin unavoidably has to be determined to plan the correct surgical procedure. Somatostatin receptor scintigraphy has been shown to be the most sensitive and widely available method for localizing the primary tumor, nevertheless >50 % of the tumors <1 cm are missed. As these tumors almost certainly will be duodenal gastrinomas, the routine use of duodenotomy with intraoperative ultrasound and transillumination of the duodenum should be performed. Pancreaticoduodenectomy should only be discussed in cases with tumor localization in the pancreatic head or in case of a persistent or recurrent tumor after previous resection [1, 28, 30–32]. In the setting of MEN1, the presence of only small (<2 cm) or no tumors in imaging studies is associated with a favorable outcome. Thus, the general recommendation to prevent malignant transformation in MEN1 patient is to confine surgery to pancreatic tumors >2 cm.