Glucagonoma syndrome is a rare paraneoplastic phenomenon. Necrolytic migratory erythema is often one of the first presenting symptoms. We report a case of a 55-year-old man with a 2-year history of recalcitrant eruption. Abdominal computer tomography was performed, which revealed a tumor in the tail of the pancreas. After pancreatectomy, his cutaneous lesions vanished in a few days. Skin symptoms are important, as they are often essential for early diagnosis of glucagonoma syndrome and may prevent metastatic disease; hence, in this report, we focus on skin disorders in glucagonoma syndrome.