Necrolytic migratory erythema (NME) is a rare paraneoplastic dermatologic condition. Its underlying cause is usually a pancreatic islet cell tumour with marked glucagon secretion. The glucagonoma syndrome is characterised by pancreatic neuroendocrine neoplasm, NME, and diabetes mellitus. We present a case of glucagonoma syndrome in a 58-year-old woman with a history of recurrent cutaneous manifestations who was referred for surgical resection of a pancreatic neoplasm after the NME was finally diagnosed. We discuss diagnostic methods, differential diagnosis, and therapeutic management of this disease.