Median arcuate ligament syndrome (MALS) is the compression of the celiac artery (CA) by the median arcuate ligament. MALS can cause pseudoaneurysm of the gastroduodenal artery, which can lead to fatal bleeding. A 40-year-old male with no prior medical history presented with symptoms of upper gastrointestinal hemorrhage (UGIH). Severe duodenal bleeding was confirmed although endoscopic hemostasis was impossible and final hemostasis was achieved following a subsequent open duodenotomy. A postoperative computed tomographic angiography (CTA) visualized a significant CA stenosis, post-stenotic dilatation and an aneurysm on a jejunal branch artery. The patient underwent coiling of the gastroduodenal artery, gastroepiploic artery and two pancreaticoduodenal arterial branches. The patient was diagnosed with MALS and 6 months later underwent open resection of the median arcuate ligament. MALS should be considered as a rare cause of upper gastrointestinal bleeding. The literature and proposed treatments are discussed.