Abstract: 　Objective　To explore the clinical feature of Kimura disease complicated with nephrotic syndrome in children. Methods　The clinical data from 4 children with Kimura disease complicated with nephrotic syndrome were retrospectively analyzed. Results　In all of the 4 male children, level of serum IgE was increased but level of eosinophils was not increased in peripheral blood. The renal pathological manifestations were different among them. Two cases had acute tubular injury, one case had pathological changes in repeated renal biopsy, and one case had a little of eosinophils infiltration in renal interstitium. All of them were sensitive to oral hormone treatment. Except that one case had no relapse yet in short follow-up period, the other 3 cases had relapsed and all manifested as positive urinary protein without lymphadenectasis. The 3 cases with recurrence were treated by combined immunosuppressive agents, the palindromia of two cases were reduced which were combined with tacrolimus. Conclusion　The Kimura disease complicated with nephrotic syndrome in children has a long course and different renal pathological manifestations. It is sensitive to hormone treatment but easy to relapse, and the treatment should be combined with immunosuppressive therapy. Tacrolimus may have the effect on reducing relapse and delaying the progress of renal pathology.