Abstract: 　Objective　To explore the clinical features, diagnosis, and treatment of childhood fibrillary glomerulonephritis (FGN). Methods　The clinical data of a child with FGN in April 2016 were analyzed retrospectively. Results　A 12-yearold boy, who presented significant proteinuria (mainly albumin), hypoalbuminemia, hypercholesterolemia, and persistent microscopic hematuria in May 2010, met the criteria of nephrotic syndrome. Renal biopsy in May 2010 showed mesangial proliferative glomerulonephritis combined with glomerulosclerosis. It was not effective by treatment with intravenous infusion of methylprednisolone and prednisolone, and there were no responses by the combination with mycophenolate mofetil and traditional Chinese medicine. After admission, the second renal biopsy was performed. Under the light microscope, the moderate mesangial proliferative glomerulonephritis combined with membranoproliferative changes was observed. Under the electron microscope, the FGN was confirmed. Conclusion　The first case of childhood FNG was diagnosed in China.