Pancreatic neuroendocrine neoplasms (pNENs) comprise a heterogeneous group of tumors. Medical managements that include biotherapy, molecular targeted therapy and chemotherapy are the main methods for patients with locally unresectable lesion or distant metastases. In recent years, the number of studies about the use of targeted antiangiogenic drugs for pNENs is growing, which provides new insight on the treatment options for this condition.