Hemophagocytic Lympho-histiocytosis (HLH) is a rare life-threatening condition characterized by widespread activation of the immune system leading to tissue damage all over the body.

Waldmann’s disease / Primary intestinal lymphangiectasia, is an unusual cause of protein losing enteropathy primarily characterized by lymphopenia, hypoalbuminemia and hypogammaglobulinemia. However, variable clinical presentation results in diagnostic dilemma and effective management. We present a toddler diagnosed with Waldmann’s disease managed with high protein diet and medium chain triglycerides supplementation.