Primary intestinal lymphangiectasia (PIL) is a rare disorder, especially in 
adults. It causes a local disruption of chylus transport and is part of the 
exudative gastroenteropathies. Conservative therapy includes dietary measures or 
somatostatin medication. Taking the differential diagnosis of PIL into 
consideration is a major challenge, since patients suffering from PIL may 
present with diarrhoea and lymphedema or chylous ascites. This can be explained 
by the chronic lymphedema of the bowel leading to dilation of the vessels 
(intraluminal loss) and sometimes even to a rupture (peritoneal loss). Push-pull 
enteroscopy and capsule endoscopy are the proper interventional diagnostic tools 
to discover PIL. Exploratory laparoscopy may be useful in unclear cases. 
Surgical resection of the altered intestine has been described with positive 
results. Exploratory laparoscopy may even be a diagnostic tool in unclear cases. 
Resection of the altered intestine is a treatment option in symptomatic and 
treatment-refractory cases.