Protein-losing enteropathy (PLE) is a clinical syndrome involving the excessive loss of proteins into the gastrointestinal (GI) tract, leading to hypoproteinemia, edema, and complications such as malnutrition, immune deficiencies, and coagulation abnormalities. PLE is caused by two main mechanisms: lymphatic abnormalities, including primary and secondary lymphangiectasia, and mucosal injury due to conditions like inflammatory bowel disease, infections, and celiac disease. Diagnosis relies on recognizing symptoms such as edema and hypoalbuminemia, with stool alpha-1-antitrypsin clearance and imaging techniques aiding in confirmation and identifying underlying causes. Management strategies focus on addressing the etiology, minimizing protein loss, and preventing complications. These include dietary modifications like low-fat, high-protein diets; supplementation with fat-soluble vitamins and trace minerals; pharmacological therapies; and surgical or interventional approaches such as lymphatic embolization. Early diagnosis and comprehensive treatment are vital for improved outcomes in children with PLE.