Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by 
protein-losing enteropathy occurring more frequently in childhood. Chronic 
diarrhea and diffuse edema are the main clinical manifestations. Peripheral 
lymphedema may also be associated. Lymphedema is usually present at the time of 
diagnosis or appears later in the course of the disease. We report the 
observation of a 31-year-old man suffering from an upper, lower limb and genital 
lymphedema many years before diagnosis of primary intestinal lymphangiectasia 
was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal 
biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and 
hypogammaglobulinemia were also noted. Treatment of lymphedema included low 
stretch bandaging and elastic stocking. No dietary management with a low-fat 
diet was added. Search for primary intestinal lymphangiectasia with biological 
parameters would be useful when primary lymphedema is present. Especially since 
primary intestinal lymphangiectasia may be complicated by occurrence of B cell 
lymphoma.