Arteriovenous malformations in the ileum are a rare cause of gastrointestinal bleeding in young adults with few reported cases and pose difficulty in diagnosing. They usually present with chronic gastrointestinal bleed. A 30-year-old woman presented with an acute episode of hematochezia with a history of intermittent melena for 1.5 years. Complete blood count revealed a low hemoglobin level of 3.5g/dl and hypochromic microcytic anemia. Oesophago-gastro-duodenoscopy was normal; however, a colonoscopy revealed the terminal ileum and colon filled with blood. Computed tomography-Angiogram showed local intraluminal contrast extravasation in the ileum. Explorative laparotomy and on-table enteroscopy were performed identifying a small elevated, pigmented, and eroded mucosa (5 to 6 mm) in proximal ileum; resection and primary anastomosis were performed. The patient was followed after surgical resection and her symptoms improved dramatically with no additional episodes of melena and with the normalization of hemoglobin.