ABSTRACT Protein-losing enteropathy is a rare complication of a variety of disorders characterized by excessive loss of protein into the gastrointestinal tract due to impaired integrity of the mucosa. Cystic hygroma is the cystic variety of congenital malformation of lymphatic system which is rare as a cause of protein-losing enteropathy (PLE). The clinical presentation of PLE is highly variable, depending on the underlying cause, but mainly consists of edema due to hypoproteinemia. We present a case of 8 year old girl presented with swelling in the right side of neck since birth and subsequently developed generalized oedema for the last one year. Her investigations showed hypoalbuminemia, pericardial effusion, ascites and cystic hygroma on lymph node biopsy and histopathology. Her upper GIT endoscopy revealed scattered white spots, having a snowflake like appearance in the mucosa of second part of duodenum, biopsy and histopathology showed diffusely dilated mucosal and sub-mucosal lymphatic vessels. Finally the case was diagnosed as a case of protein losing enteropathy due to Cystic hygroma. Keywords: Cystic hygroma, Protein losing enteropathy, lymphangiectasia