c w t A woman was referred to our department for a 20-year history of recurrent lower-limb edema and interittent diarrhea with mild intestinal symptoms that had proressively worsened during the past 6 months, accounting for sthenia and weight loss. Routine blood tests performed on a early basis invariably showed mild anemia and severe hyoalbuminemia, and the patient thus had received periodic ourses of parenteral albumin supplementation. Despite the umber of instrumental examinations performed, including pper and lower gastrointestinal endoscopies, no conclusive iagnosis had been made. Indeed, normal mucosa was found at istologic examination of all biopsy specimens, and only a mild all thickening of the jejunal loops was evident by computed omography. Furthermore, she had an unremarkable family istory, and had not developed any comorbidity over the past ears. At admission, severe hypoproteinemia (5.0 g/dL) with hypolbuminemia (2.4 g/dL) were confirmed, and marked lymhopenia (472 cells/ L) and hypo-globulinemia (immunoglobulin [Ig]G, 423 mg/dL; IgA, 158 mg/dL; and IgM, 35 mg/ dL) also were found. Both upper and lower endoscopic examinations with intubation of the terminal ileum were performed with the appearance of normal features even at histologic evaluation of mucosal specimens. Computer-aided tomography enteroclysis (Figure A, axial image) showed sigificant thickness of both small-bowel loops (solid arrows) nd mesentery (solid arrowheads), with an increased size of all lood vessels (empty arrowheads), and remarkable luminal lling defects in the dilated loops (empty arrows). Oral doule-balloon enteroscopy therefore was performed, showing cattered whitish multiple circumferential nodular and polp-like lesions starting from the proximal jejunum (Figure B, olid arrows), where the folds were still recognizable even hough they were fully edematous (Figure B, empty arrows), to he proximal ileum where they were uncountable and comletely replaced the mucosal profile (Figure C, empty arrows) to he extent that the intestinal lumen was considerably narrowed, nd the gut wall was so rigid that it was impossible to insert the nstrument any farther. Histologic evaluation of multiple erendoscopic biopsies revealed remarkable dilation of lymhatic spaces mainly in the pericryptal zones and lamina proria (Figure D, white and black arrows, respectively), consistent ith a diagnosis of intestinal lymphangiectasia. Possible causes f secondary lymphangiectasia, that is, Whipple disease, intesinal tuberculosis and other granulomatous conditions, lymhomas and other intra-abdominal malignancies, retroperitoeal fibrosis, and cardiac failure, were excluded, and the disease, herefore, was defined as primary lymphangiectasia. The patient as discharged with the prescription of a low-fat, high-protein iet with oral supplementation of medium-chain fatty acids. At he 6-month follow-up visit, no clinical signs of disease recurence were evident, with a critical improvement of laboratory est results. A complete recovery of serologic parameters was chieved after 12 months of specific dietetic treatment. Intestinal lymphangiectasia is a rare pathologic condition haracterized by dilated lymphatic vessels in the intestinal all and small-bowel mesentery inducing protein-losing eneropathy and lymphocyte depletion.1 The disease is characterized by patchy lesions, often making diagnosis difficult because of their localization in the jejunoileum.2 Double-