A 61-year-old female presented with progressive glucagonoma of a pancreatic neuroendocrine tumor, accompanied by necrolytic migratory erythema. She had previously undergone surgical resection, octreotide treatment, transarterial embolization, and chemotherapy featuring epirubicin, 5-fluorouracil, and dacarbazine, over a period of 8 years. Her necrolytic migratory erythema status correlated with the extent of disease control. However, her glucagonoma and necrolytic migratory erythema did not respond to everolimus; therefore, we started sunitinib (37.5 mg/day) treatment in an effort to inhibit vascular endothelial growth factor- and platelet-derived growth factor-mediated receptor signaling. Four months later, magnetic resonance imaging revealed that the liver metastasis had responded to this treatment, associated with improvement of the necrolytic migratory erythema. The serum glucagon level (initially 803 pg/mL; reference level 71-174 pg/mL) fell to 425 pg/mL. Successful treatment of glucagonoma should improve necrolytic migratory erythema. Here, we report the first case of necrolytic migratory erythema developing secondarily to metastatic glucagonoma, and exhibiting sensitivity to sunitinib.