Primary intestinal lymphangiectasia (PIL) is rare disorder characterized by 
congenital malformation or obstruction of intestinal lymphatic drainage; it is 
responsible for protein losing enteropathy leading to lymphopenia, 
hypoalbuminemia and hypogammaglobulinemia. A low-fat diet associated with 
medium-chain triglyceride supplementation is the cornerstone of PIL management. 
The administration of intravenous immunoglobulins does not always lead to 
satisfactory plasma levels and therefore the replacement therapy with 
immunoglobulins is controversial. We describe here the case of a patient with 
PIL and severe hypogammaglobulinemia treated with immunoglobulins. The striking 
aspect of this case is the clinical and serological benefit obtained with the 
subcutaneous compared to the intravenous immunoglobulins administration.