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Pancreatic arteriovenous malformation (PAVM) is defined as a vascular anomaly with an abnormal anastomosis of the arterial and portal networks within the pancreas. Treatment modalities of PAVM include transarterial embolisation (TAE), irradiation and operation. Most patients treated with TAE alone will experience recurrence, so surgery is the best radical treatment. A female patient was admitted to our institution for the treatment of haematemesis. Examination revealed varices in the oesophagus and stomach, collateral circulation development caused by portal hypertension and PAVM of the pancreas. Surgical treatment was intended to reduce left portal hypertension. In this case, collateral circulation were considered dangerous points for unexpected bleeding. TAE was performed on the splenic artery before surgery to reduce blood flow in the areas with collateral circulation. En bloc resection of retroperitoneal tissue using the surgical procedure of radical antegrade modular pancreatosplenectomy was effective to minimise blood loss. [ABSTRACT FROM AUTHOR]