Extrapulmonary small-cell neuroendocrine cancers are rare in clinical practice. They are frequently metastatic at the time of diagnosis with survival in months even with the most intensive treatment. So far, treatment recommendations for this group rely on data from the similar but more common small-cell carcinoma of the lungs. Immune checkpoint inhibitors are being investigated for the treatment of metastatic small-cell lung cancer with positive outcome. We applied the experience from these studies to a case of metastatic small-cell neuroendocrine cancer of the pancreas using nivolumab as a treatment of last resort. We noted a favorable and durable response suggesting that this may be superior to all currently available options for palliative treatment in a similar scenario.