Upper gastrointestinal haemorrhage is a major medical emergency and accounts for approximately 7,000 admissions to hospitals in Scotland each year. Over the last 10 years there has been a number of improvements in diagnosis and conservative management of the condition, which significantly reduced the ratio of life-threatening cases requiring an emergency surgery. Despite these achievements surgical intervention or, if accessible, endovascular procedures must be undertaken as emergency actions, should conservative management fail. Vascular malformations of the duodenum are less frequent causes of upper GI bleeding. Duodenal varices found endoscopically occur in 0.4% of patients with portal hypertension (PHT) and are believed to be caused mainly by liver cirrhosis, idiopathic PHT, extrahepatic PHT, or previous surgical trauma. The duodenal bulb is their most common site, followed by the second portion of the duodenum. Forty per cent of patients with PHT have duodenal varices at angiography; however, their penetration unusually affects submucosa, hence no symptoms develop. Isolated bleeding duodenal varices are scarcely reported in literature, although present a significant surgical problem: massive haemorrhage combined with failure to identify them as a source has led to catastrophic outcomes with mortality rate of 40%. The case hereby presented is unique in several aspects. Duodenal varices were explored on emergency laparotomy rather than on prior endoscopies, which, performed by the same well-established endoscopists, were twice negative. This corresponds to the study by Cottam et al. stating that duodenal varices may not penetrate the submucosa, hence haemorrhages of their origin may even be more difficult to diagnose on endocsopy. Secondly, the haemorrhage here reported was undoubtedly a life-threatening condition that required a multidisciplinary team to be managed successfully. Along with Shirashi et al. we confirm that surgical ligation followed by the excision of duodenal / small intestinal varices may be an effective method of their management - both cases have been free of recurrence at 15 months postoperatively. In contrast to the study by Hashizume et al. the duodenal varices here presented were not associated with portal hypertension (PTH). Finally, duodenal varices located in the posterolateral aspect of the descending duodenum are less common as the majority of cases reported so far were of duodenal bulb location.