Pancreatic neuroendocrine neoplasms are rare tumors with low response rates to anti-programmed cell death protein-1 immunotherapy Nivolumab combined with low-dose ipilimumab demonstrated a clinical benefit rate (includes stable disease >6 months plus objective response) of 26% with durable benefit lasting >2 years in 3 of 19 patients. These results suggest that dual immunotherapy may be useful for a subset of patients with this rare cancer and that additional investigations are warranted.