Primary intestinal lymphangiectasia (PIL), so-called Waldmann's disease, is an 
uncommon condition, characterized by dilated intestinal submucosal and 
subserosal lymphatics of the gastrointestinal tract. Protein-losing enteropathy 
is the most common manifestation of this supposed congenital disease. Since the 
initial description in 1961, 11 cases of lymphoma have been reported suggesting 
that PIL predisposes to lymphoma. Here, we report the first case of primary 
nodal location lymphoma during PIL with recovery of the protein-losing 
enteropathy after its treatment by radiochemotherapy.