Objective　To discuss the clinical characteristics and prognosis of lipoprotein glomerulopathy (LPG) in children. Method　Clinical data of one pediatric LPG patient were retrospectively analyzed. The clinical features and prognosis of childhood LPG were summarized based on literature review. Results　A nine years old girl presented with frequent urination. The first urine test revealed hematuria and proteinuria. After one week anti-infection treatment, the hematuria and proteinuria were continued. The serum albumin was slightly reduced. The hyperlipidemia and mild anemia were emerged. Kidney biopsy showed that enlarged glomeruli, with dilated capillary loops and weak eosinophilic lipoprotein thrombi in the capillary lumina under the light microscope; layered or tufted "embolus" containing particulated lipid vacuoles under electron microscope. Gene sequencing identified APOE Tokyo (Leu141-Lys143→0). The diagnosis of LPG was confirmed. The lipid-lowering therapy was administrated and the disease was alleviated. Conclusion　LPG is a rare disease in children. The level of blood lipid was significantly increased, and the hormone therapy was ineffective. Kidney biopsy is the main basis for diagnosis. The genetic testing can prompt the genetic background. Lipid lowering therapy can relieve the progress of the disease.