Primary intestinal lymphangiectasia (PIL) is a protein-losing, exsudative 
gastroenteropathy causing lymphatic obstruction. Diagnosis depends on clinical 
examination and histological findings. Conservative treatment modalities include 
a low-fat diet and enteral nutritional therapy in order to reduce enteric 
protein loss and to improve fat metabolism. Other treatment options consist of 
administration of antiplasmin or octreotide to lower lymph flow and secretion. 
We report on a 58-year-old patient who underwent exploratory laparotomy due to a 
worsening physical status, recurrent chylaskos and leg oedema under conservative 
dietary therapy. Intraoperative findings showed a typical PIL of the jejunum 
about 20 cm distal to the Treitz's ligament. Histological examinations confirmed 
this diagnosis. One year after segmental small bowel resection (105 cm) with 
end-to-end anastomosis the patient is healthy, free of symptoms, has gained 
weight and his serum protein level has increased. Intraabdominal ascites and leg 
oedema have not reoccurred since.