Intestinal lymphangiectasia, characterized by dilatation of intestinal lacteals, is rare. The major treatment for primary intestinal lymphangiectasia is dietary modification. Surgery to relieve symptoms and to clarify the etiology should be considered when medical treatment failed. This article reports a 49-year-old woman of solitary duodenal lymphangiectasia, who presented with epigastralgia and anemia. Her symptoms persisted with medical treatment. Surgery was finally performed to relieve the symptoms and to exclude the existence of underlying etiologies, with satisfactory effect. In conclusion, duodenal lymphangiectasia can present clinically as epigastralgia and chronic blood loss. Surgical resection may be resorted to relieve pain, control bleeding, and exclude underlying diseases in some patients.