目的　探讨儿童脂蛋白肾病(LPG) 的临床及预后。方法 回顾性分析1 例儿童LPG 的临床资料，归纳总结国内外报道的儿童LPG 的临床特点及预后。结果　患儿，女，9 岁，以尿频起病，初次尿检提示菌尿、血尿、蛋白尿，规律抗感染治疗1 周后，仍有血尿、蛋白尿，血清白蛋白轻度降低，高脂血症，轻度贫血；肾脏组织活检，镜下可见肥大肾小球，扩张的肾小球毛细血管管腔，其内充以脂蛋白栓子，油红O 染色阳性；电镜下多见层状或簇状“栓子”内含颗粒状脂质空泡。基因检测APOE Tokyo (Leu141-Lys143 → 0)。诊断为LPG，给予降脂治疗后病情明显缓解。结论 儿童LPG 罕见，血脂水平显著增高，激素治疗无效，肾脏穿刺活检是确诊的主要依据，基因检测提示其遗传背景；降脂治疗可缓解病情进展。

　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.