Vascular malformations of the small bowel are uncommon, yet remain a cause of gastrointestinal bleeding in up to 5% of patients with gastrointestinal hemorrhage. The diagnosis of these lesions is suspected in patients with a gastrointestinal source of blood loss with a negative upper endoscopy and colonoscopy. Enteroscopy remains the mainstay in diagnosing these lesions. Therapeutic options include endoscopic coagulation, pharmacologic agents (hormones, octreotide, iron replacement), and occasionally surgery. Pharmacologic and endoscopic treatments are discussed.