Objective To explore the clinical application and efficacy in the treatment of children with bilate-ral Wilms tumor(BWT) by retroperitoneal nephrectomy or transabdominal nephrectomy. Methods Clinical data of 27 children with BWT in which 9 cases underwent retroperitoneal nephrectomy (study group) while 18 cases underwent transabdominal nephrectomy (control group) at Department of Pediatric Surgery, the First Affiliated Hospital of Zhengzhou University from April 1997 to July 2014 were retrospectively analyzed.The perioperative clinical parameters and survival data were compared between the study group and the control group. Results The operation duration [(129.5±13.9) min], the length of intensive care unit (ICU) postoperatively [(6.3±3.1) d], hospital stay[(13.9±3.9) d] and the time to resuming an oral diet[(6.8±3.4) d] of the study group were markedly lower than those of the control group [(149.5±26.7) min, (9.7±3.8) d, (20.5±3.0) d, (11.5±3.1) d], and the diffe-rences were statistically significant (t=2.129, P=0.051; t=2.551, P=0.017; t=4.489, P<0.001; t=3.488, P=0.002). The estimated blood loss [(95.5±14.6) mL], postoperative renal recovery time [(22.3±3.1) d] and mean serum creatinine levels by follow-up [(67.0±21.3) μmol/L] of study group were lower than those of the control group [(105.9±23.1) mL, (24.7±4.5) d, (76.8±13.3) μmol/L], but the differences were not significant (t=1.231, 1.485, 1.474, P=0.243, 0.163, 0.154). There was a similar tendency that intraoperative adverse event rates [0(0/9 cases)] and postoperative urine leakage rates [11.1%(1/9 cases)] of the study group were lower than those of the control group [11.1%(2/18 cases), 16.7%(3/18 cases)], but the differences were not significant (P=0.103, 0.093). Study group revealed longer median survival time than that of the control group and cumulative survival hazard of study group was lower than that of the control group (28.5 months, 95%CI: 23.2-33.7 months vs 22.4 months, 95%CI: 12.6-32.1 months; χ2=0.469, P=0.494). Conclusions Retroperitoneal nephrectomy provides valuable benefits clinically, including shorter operative time, postoperative length of ICU and hospital stays, avoidance of bowel injuries, and less adhesions and shorter time to resuming an oral diet over the transabdominal counterpart, which can be considered a viable surgical option for treatment of BWT. Key words: Retroperitoneal nephrectomy; Transabdominal nephrectomy; Bilateral Wilms tumor; Child