Pancreatic neuroendocrine tumors (PNETs) are rare malignancies that arise from the islets of Langerhans. The role of standard chemotherapy in advanced well-differentiated PNETs remains to be defined. Sunitinib is an oral multitargeted inhibitor with antiangiogenic and antitumor properties that has shown significant improvement in survival in metastatic PNETs, although objective responses by Response Evaluation Criteria in Solid Tumors were only 9%. We herein report on the case of a middle-aged woman with metastatic PNET who was heavily pretreated for her advanced disease with limited success, and who showed clinical, biochemical, and radiological responses by using Choi criteria but not Response Evaluation Criteria in Solid Tumors criteria. To our knowledge, this is the first reported case of treatment with sunitinib in a patient with PNET in response to Choi criteria.