Objective　To explore the clinical features and prognosis of primary intestinal lymphangiectasia (PIL). Methods　The clinical data from one pediatric patient with PIL was retrospectively analyzed. The relevant literatures were reviewed. Results　The 5-year-old female patient gradually presented with progressive abdominal distension, diarrhea, and general edema after birth. Laboratory tests revealed hypoalbuminemia, lymphocytopenia, and hypogammaglobulinemia. Gastroscopy showed diffuse white spots in duodenum and jejunum. The histopathology confirmed greatly dilated lymphatics in interstitial mucosa. After diagnosis, the intravenous infusion of albumin, diuretic, and dietary adjustment were administrated. The patient quickly relieved and was discharged. Conclusions　PIL is a rare protein-losing enteropathy. The diagnosis of PIL mainly relies on small intestine biopsy. Early diagnosis, early dietary intervention and parenteral nutrition support could improve the symptoms and signs.