Objective To evaluate the safety and efficacy of laparoscopic partial nephrectomy(LPN)for multilocular cystic renal neoplasm(MCRN)in children.Methods The clinical data of a case with MCRN admitted to Wuhan Children's Hospital affiliated to Tongji Medical College of Huazhong University of Science and Technology in August 2020 was retrospectively analyzed.A 12-year-old boy was found with a left cystic renal mass and enlarged by ultrasound 1 year before admission.Ultrasound and CT examination showed a 5 cm MCRN in the lower pole of left kidney.The enhanced CT showed that the mass was about 10 HU,which showed septation and punctate calcification,and a wall thickness of one separated enhancement was>4 mm.A preoperative diagnosis of left renal cystic lesion(grade Bonsiak Ⅲ,stage T1bN0M0,R.E.N.A.L.score 9X).The lesion had an intermediate probability of being malignant.The patient underwent a laparoscopic left partial nephrectomy.During the operation,there was a 60 mm × 50 mm cystic mass in the lower pole of left kidney,which was removed intact.Results The pathological report of the mass was MCRN.Its morphological features showed multilocular cystic changes.No tumor component was found in its perirenal adipose tissue and incisional margin of its calyces.The expression of TFE3 was detected by immunohistochemistry and fluorescence in situ hybridization.It is consistent with renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion(Xp11.2 tRCC).After 3 years of follow-up,the shape of the left kidney was recovered partially without recrudescence and distant metastasis.His serum creatinine was 61.6 μmol/L.Conclusions According to Bosniak classification of cystic renal masses(version 2019),benign and malignant MCRN in children were predicted.LPN is a safe and effective minimally invasive treatment for MCRN in children,which can preserve part of the renal function and improve the quality of life after operation.