BackgroundLymphangiomas are a heterogeneous group of congenital vascular malformations characterized by cystic dilation of lymphatic vessels. They can occur at any age, but they are more common during childhood and in cutaneous localizations. Hemangiomas and vascular malformations of the gastrointestinal tract are very uncommon. Most are asymptomatic and diagnosis is often made as an incidental finding during endoscopy. On rare occasions the initial manifestation can be chronic anemia due to low grade gastrointestinal bleeding. They constitute an unusual manifestation and there is a low incidence of this type of tumor.Case presentationWe report the case of a 43 year-old latin female, with a 2-year history of chronic anemia requiring blood transfusion. Hemoglobin and Hematocrit count were low, therefore further studies were required to rule out bleeding sources or other causes of anemia. Enteroscopy findings showed a 35 mm lesion taking up 50 % of the circumference in the distal duodenum, with raised whitish edges secondary to confluent lymphangiectasia, a center with a vascular appearance and active bleeding spots. Biopsy samples dyed with India ink confirmed the diagnosis of hemangiolymphangioma.ConclusionDiagnostic difficulties in this case, highlight the need to include hemangiolymphangioma in the differential diagnosis of chronic anemia as well as the need for multiple diagnostic methods to confirm the presence of the condition.