Glucagonoma is a rare endocrine tumor of the pancreas. We report curative resection for malignant glucagonoma in 60-year-old Japanese man admitted to our department because of hyperglycemia and a pancreatic tumor (detected at another institution). On admission, his serum glucagon level was 580pg/ml. Computed tomography (CT) showed a tumor measuring approximately 2cm in diameter, in the head of the pancreas. Angiography revealed a hypervascular lesion fed from the gastro-duodenal artery. Endoscopic retrograde cholangiopancreatography (ERCP), revealed a stenosis of the pancreatic duct in the head of the pancreas and dilation of the caudal side, with indications that the tumor had invaded the pancreatic duct. The tumor was diagnosed as a glucagonoma in the head of the pancreas. Pancreaticoduodenectomy was successfully performed. In the resected specimen, the tumor, which was well defined within the peripheral parenchyma of the pancreas, measured 3.3 • 2.5 • 2.0cm. The clinico-pathological stage of the tumor on pTNM classification was Stage III (pTlb, pN1, pM0). Although the prognosis for malignant glucagonoma is very poor because distant metastasis is frequently present when the tumor is diagnosed, and because of the hormonal effects of the tumor, the patient was well when discharged, without any complications, and no recurrence has been detected in the 6 years since the operation.