Protein-losing enteropathy is a rare condition character-ised by protein loss through the intestines, which leads to hypoproteinemia. It may result from various causes, including primary intestinal lymphangiectasia, amyloido-sis, rheumatic diseases, parasitic infections, or tumours. Primary intestinal lymphangiectasia is a congenital dis-ease where lymph leaks into the intestines due to dilated lymphatic vessels. It usually appears within the first three years of life; however, even though it is rare and mostly sporadic, some genetic and syndrome associations were studied. Main symptoms include oedema, weight loss, intermittent diarrhoea, nausea and vomiting. Diag-nosis is based on radiological, endoscopic and histologi-cal findings. Treatment involves nutritional support, medications (immunosuppressants, diuretics, antifibrino-lytics) and, in some cases, surgery. This report presents a case of primary intestinal lymphangiectasia in a seven-year-old child.