Necrolytic migratory erythema (NME) is a classical paraneoplastic symptom observed in patients with pancreatic glucagonoma. We report a 46-year-old Japanese woman with glucagonoma who presented with mucocutaneous manifestations 1 year prior to the diagnosis of the pancreatic neoplasm with multiple liver metastases. She was treated with octreotide long-acting release, a somatostatin analog, which resulted in a dramatic improvement of NME within 2 weeks after the start of treatment. Increased awareness of NME may avoid unnecessary delay in the diagnosis of pancreatic glucagonoma.