This article describes two new cases of primary intestinal lymphangiectasia: the first one refers to a 10-years-old girl, who failed to thrive, while the second one was discovered in a 19-months-old female infant, who presented with anasarca. In both cases gastroduodenal endoscopy allow us to put the diagnosis, revealing the presence of the typical duodenal lesions, due to dilatation of the lymphatic vessels and loss of the lymph. The typical endoscopic features found in both cases are here described; at the same time, the major role of the intestinal biopsy via endoscopic route is outlined, since this is the only method which allow to perform targeted biopsies.