Abstract: 　Objective　To explore the correlation between clinical and pathological features of Wilms tumor in children. Method　The clinical and pathological data in 67 cases of pathologically confirmed Wilms tumor were retrospectively analyzed. Results　In the 67 children, 37 were male and 30 were female. The percentage of children with age at onset under 5 years is 86.6%. The clinical manifestations included abdominal mass in 25 cases (37.3%), hematuria in 17 cases (25.4%) and abdominal pain in 13 cases (19.4%). All the children had unilateral onset, and 64 cases (95.5%) had tumor diameter greater than 5 cm. Lymph node metastasis and distant metastasis were rare, 5 cases (7.5%) and 14 cases (24%) respectively. The common metastatic sites included lung, liver, etc. The children were pathologically divided into good prognosis tissue group (FH) and poor prognosis tissue structure (UFH) group, 63 cases (94.0%) and 4 cases (6.0%) respectively. There was no difference in age, sex, clinical manifestations, tumor location, tumor diameter, lymph node metastasis and distant metastasis, P53 positive rate and expression rate of Ki-67 ≥30% between FH group and UFH group (all P>0.05). There was no significant correlation among sex, age, tumor location, tumor diameter, histological type, lymph node metastasis, recurrence and metastasis (all P>0.05). The expression of Ki67 and P53 was significantly different between genders (all P<0.05). The tumor diameter and tumor location had no correlation with lymph node metastasis, recurrence, metastasis and Ki-67 expression in children (all P>0.05). The expression of P53 between different tumor diameters and between different tumor locations were statistically different (all P<0.05). Conclusion　Wilms tumor has some clinical and pathological characteristics such as large tumor size, unilateral, less invasiveness, better prognosis and more sensitive to preoperative radiotherapy and chemotherapy. Complete surgical resection combined with chemotherapy is the main treatment.