Purpose: The aim is to expose the atypical presentation in an early diagnosed glucagonoma case. Method: A 76-year-old man with altered fasting basal glucose levels and a constitutional syndrome of 4 months of evolution, associated with palpitations and pigmented and pruritic skin lesions. Abdominal CT: 14mm hyperdense nodular structure in the pancreatic tail with calcifications. Hormonal study: glucagon> 400 pg / ml. Neuronal specific enolase and CgA positive. Negative tumor markers. Ultrasound-guided FNA: neuroendocrine tumor, glucagonoma. PET-CT: negative for metastasis. Octreoscan: pancreatic injury. Surgical treatment is decided: distal splenopancreatectomy and lymphadenectomy. Results and conclusions: Glucagonoma classically presents with weight loss and the “4 Ds”: diabetes, dermatitis (necrolytic erythema migrans), deep vein thrombosis, and depression. In our case, the presentation is atypical, with little weight loss, skin lesions other than necrolytic migratory erythema and altered fasting blood glucose levels. Are we changing the natural history of these tumors due to their early diagnosis with the increasingly requested imaging? This raises us whether in the future we should suspect them of atypical forms of presentation and whether new therapeutic strategies will be considered as a result of their early diagnosis. Currently, surgical treatment is indicated from the beginning in all sporadic functioning pancreatic neuroendocrine tumors (there are some exceptions in cases of genetic syndromes) and resectable. The surgical indication is for cephalic duodenopancreatectomy or distal splenopancreatectomy depending on the location of the tumor, associated with systematic lymphadenectomy due to its high rates of malignancy and lymph node involvement.