<h4>Background</h4>Tumor-associated hypoglycemia can be caused by non-islet cell tumors including gastrointestinal stromal tumor (GIST) which is a rare paraneoplastic syndrome that leads to the release of insulin-like growth factor-2 (IGF-2).<h4>Case presentation</h4>We report the case of a 45-year old woman who was admitted to our hospital with refractory hypoglycemic episodes. The gene discussed is IGF2; the disease is pancreatic neuroendocrine tumor.