INTRODUCTION: Intestinal lymphangiectasia (IL) is a rare disease characterized by dilated lymphatic vessels in the intestinal wall and small bowel mesentery, which induce loss of protein and lymphocytes into the bowel lumen. Gastrointestinal bleeding (GIB) rarely has been described as the only symptom of IL. We describe a case of obscure GIB in a young female caused by lymphangiectasia localized to the distal jejunum by antegrade double balloon enteroscopy. CASE DESCRIPTION/METHODS: A 41-year-old African American female who initially presented to her PCP with chronic fatigue and intermittent episodes of melena. The patient was subsequently placed on iron supplementation and referred to gastroenterology for further work up. Physical examination finding were largely unremarkable. On laboratory tests, her hemoglobin declined to 5.8 g/dL (from a baseline of 10 g/dL). Initial Esophagogastroduodenoscopy (EGD) showed 2 cm hiatal hernia but was otherwise unremarkable. A subsequent colonoscopy showed mild internal hemorrhoids and a 3 mm solitary ulcer in the distal rectum but no other obvious sources of GI bleeding. Magnetic resonance enterography (MRE) was unremarkable. Next, a video capsule endoscopy was done which showed a site of active bleeding from the mid-small bowel and was associated with nodular mucosa and prominent lacteals. A subsequent double balloon enteroscopy (DBE) showed an oozing fold in the distal jejunum with white speckled appearance consistent with a bleeding lymphangiectasia. A total of seven clips were placed on the lesion and during application of clips, a white milky substance was noted coming from the lesion suggesting the diagnosis of lymphangiectasia. DISCUSSION: IL is most commonly found in the small bowel, making its diagnosis difficult by EGD or colonoscopy. DBE and video capsule endoscopy (VCE) are the two most important diagnostic modalities available to evaluate the entire small intestine.The noninvasive nature of VCE and its capability to visualize the entire small bowel has made it a first-line tool in many centers. However, DBE is preferred over video capsule endoscopy because of higher sensitivity from better endoscopic visualization and ability to perform biopsies for pathologic diagnosis. In this report, we present an extremely rare case of primary intestinal lymphangiectasia in causing obscure GIB and successful intervention using DBE. We believe this is the second case to report small bowel bleeding from jejunal lymphangiectasia, which was successfully treated using DBE.