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Outcome of children with Wilms tumor (WT) has improved remarkably because of the advancements in Pediatric Oncology. However, in most developing countries survival rates are still poor. There is a paucity of data regarding the clinical profile and outcome of children with WT in India.Children with WT who were treated at our center between 2005 and 2014 were retrospectively analyzed.Over the 10-year study period, 108 children with WT were treated in our unit. The median age at presentation was 30 months (male:female ratio of 4:1). Abdominal mass was the most common presenting feature (91%). The majority of children had stage 2 (30%) or stage 3 (35%) disease. Fifty-nine children had large or bilateral tumors requiring preoperative chemotherapy. Of the total 108 children, 11 died during primary treatment and 14 relapsed after completion of treatment. The projected 5-year overall survival is 74%, whereas the event-free survival is 73%.Children with WT in our setting present late with advanced disease and large tumors, which are technically difficult to deliver at surgery. With a multidisciplinary approach, reasonably good survival rates were achieved even in the resource-limited Indian scenario.