Necrolytic migratory erythema is considered an obligatory cutaneous paraneoplastic sign associated with glucagonoma. Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumor of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema. Although necrolytic migratory erythema is a specific finding in glucagonoma syndrome, it may occur in other settings, unassociated with an alpha-cell pancreatic tumor (pseudoglucagonoma syndrome). The rarity of glucagonoma imposes a challenge, with most patients being diagnosed after a long period of treatment for their skin rash. The main prognostic sign of glucagonoma are the subsequent metastases that come late in the course of the disease. Herein, we present a 55-year-old female patient with a 5-year history of unrecognized cutaneous and systemic manifestations of glucagonoma syndrome. Based on the investigations, the diagnosis of glucagonoma syndrome without metastases was established. After surgical removal of pancreatic carcinoma/glucagonoma, complete healing and a long disease-free period was achieved. Appropriate awareness of the characteristics of necrolytic migratory erythema in physicians/dermatologists often leads to an early diagnosis of glucagonoma syndrome and enhances the chances of a favorable outcome.healing and long disease-free period was achieved. The awareness of physicians/dermatologists of the characteristic necrolytic migratory erythema, often leads to an early diagnosis of glucagonoma syndrome and enhance the chances of a favorable outcome.</p