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Volume 2 -Issue -5 Case PresentationPrimary intestinal lymphangiectasia (PIL) is a rare cause of obscure gastrointestinal (GI) bleeding that may range from chronic blood loss to massive bleeding. Few reports have been described in adults [1][2][3][4] and very few in children [5]. Here, we describe a case of an infant diagnosed with PIL who presented with severe iron deficiency anemia and hypoalbuminemia, in order to demonstrate the importance of considering abnormalities of the lymphatic system as a cause of anemia.A 1 year-old child presented to Buzzi children hospital in October 2017 with 2 months of diffuse edema, pallor and weakness; his growth in weight was stable on the 10 th -25 th centile; otherwise his medical history was unremarkable. Physical examination revealed significant pallor and swelling with anasarca. In particular he presented with periorbital, hands, scrotal, sacral, feet and limb-pitting edema. Moreover, cardiac examination revealed high heart rate and an unknown systolic 2/6 murmur. Upon primary evaluation, laboratory tests showed severe irondeficiency anemia (hemoglobin 3,8g/dl, mean corpuscular volume 57,3fl, ferritin 1µg/l, reticulocytes 0,9%), hypoalbuminemia (2g/ dl) and hypogammaglobulinemia (0,1g/dl). No proteinuria was found in his urinalysis. Abdominal ultrasound revealed ascites; no alterations were found on chest X-ray or echocardiography. Fecal occult blood was highly positive.In the hypothesis of an enteropathy we first considered celiac disease as a diagnosis, however specific celiac type serology was negative and upper gastrointestinal endoscopy and histology were unremarkable. On ileo-colonoscopy no alteration was found. A 99mT-DTPA scintigraphy showed an abnormal diffusion of the radioisotope in the intestinal lumen in particular in the small bowel and suggested a diagnosis of a protein losing enteropathy Therefore,