BACKGROUND: Pancreatic endocrine tumors are uncom-mon neoplasms and can lead to systemic disorder includingglucagonoma syndrome, a very rare prototypical paraneo-plastic phenomenon. The aim of this study was to assess thediagnosis and surgical strategy for the treatment of glu-cagonoma syndrome.METHODS: The clinical data of a case of pancreatic headtumor with typical glucagonoma syndrome of necrolyticmigratory erythema (NME), diabetes mellitus (DM), ane-mia, and glossitis were retrospectively analyzed.RESULTS: Cutaneous eruption occurred mainly in thegroin, extremities, thighs, buttocks, and perineum. Ahighly elevated level of serum glucagon was detected by ra-dioimmunoassay. A tumor located in the head of the pan-creas was well-defined by pre- and intra-operative ultra-sonography, contrast enhanced computed tomography,and magnetic resonance imaging. Tumor enucleation wasperformed, showing significantly improved symptoms.Near complete resolution of NME was shown one week af-ter surgery. Surgical complications or recurrence was notfound.CONCLUSIONS: The diagnosis of glucagonoma syndromeis established by marked clinical features such as NME asthe hallmark clinical finding, hyperglucagonemia, and ra-diographically demonstrated neuroendocrine tumor. Thetopographic diagnosis of glucagonoma can be achieved bycombined imaging methods. Enucleation of tumor is a va-luable treatment for solitary pancreatic tumor withoutperipancreatic invasion, liver metastasis, and pancreaticduct compression. BACKGROUND: Pancreatic endocrine tumors are uncom-mon neoplasms and can lead to systemic disorders including glucagonoma syndrome, a very rare prototypical paraneo-plastic phenomenon. The aim of this study was to assess the diagnosis and surgical strategy for the treatment of glu-cagonoma syndrome. METHODS : The clinical data of a case of pancreatic head tumor with typical glucagonoma syndrome of necrolytic laboratory erythema (NME), diabetes mellitus (DM), ane-mia, and glossitis were retrospectively analyzed .RESULTS: Cutaneous eruption occurred mainly in the groin, extremities, thighs, buttocks, and perineum. Ahighly elevated level of serum glucagon was detected by ra-dioimmunoassay. A tumor located in the head of the pan-creas was well-defined by pre- and intra-operative ultra-sonography, contrast enhanced computed tomography, and magnetic resonance imaging. Tumor enucleation wasperformed, showing significantly more symptoms. Near complete resolution of NME was shown one week af-ter surgery . Surgical complications or recurrence was not found. CONCLUSIONS: The diagnosis of glucagonoma syndrome established by marked clinical features such as NME asthe hallmark clinical finding, hyperglucagonemia, and ra-diographically demonstrated neuroendocrine tumor. Thetopographic diagnosis of glucagonoma can be achieved bycombined imaging methods. of tumor is a va-luable treatment for solitary pancreatic tumor withoutperipancreatic invasion, liver metastasis, and pancreatic duct compression.