IntroductionHigh-grade pancreatic neuroendocrine tumors are rare and most of them are well-differentiated. Management of this type of tumor is not yet well established.MethodsA 28-year-old women was referred to our hospital for a well-differentiated grade 3 pancreatic neuroendocrine tumor with multiple liver metastases. The patient received neoadjuvant therapy consisting of combination of Capecitabine/Temozolomide chemotherapy and somatostatin analogue.ResultsAn excellent regression was observed on both pancreatic and hepatic lesions and therefore an aggressive surgical management could be performed with a two-step scheme. Adjuvant hormono-chemotherapy was administered between the two surgeries. The patient achieved six years of disease-free survival following the last therapy.Discussion and conclusionWe highlight the difference between well and poorly differentiated high grade neuroendocrine neoplasia and report that aggressive surgery is a valid option even in metastatic presentation at diagnosis.