Introduction: Wilms’ tumor or nephroblastoma is an embryonal tumor of childhood. Wilms tumor being the most common primary renal malignancy in children accounts for 5% of childhood malignancies. This study aimed to assess the outcome of Wilms tumor in children who underwent nephroureterectomy after neoadjuvant chemotherapy and evaluate the influence of the stage of disease on treatment outcomes. Methods: This was a retrospective observational study conducted in the Department of Paediatric Surgery, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh during the period from January 2012 to July 2015. In our study, we included 30 pediatric patients diagnosed with Wilms tumor who underwent nephroureterectomy at our institution. Result: We found the mean age was 39.5 ± 14.3 months. The majority (63%) of our study patients were male compared to female (37%). Of all 30 patients, 40% were diagnosed with low-stage (stage I to II) disease, and 60% were diagnosed with high-stage (stage III to stage V). The patients with low-stage (stage I to II) disease had a 100% survival rate, whereas the patients with high-stage (stage III to stage V) disease had a 44% survival rate. Among all patients, 22(73%) patients had favorable histology, while 8(27%) patients had unfavorable histology. Conclusion: In our study, the postoperative results were uneventful and the recurrence of symptoms or any other complaints was not observed in any case. The earlier stage of the disease and favorable histology had a higher chance of cure compared to the late stage of the disease or unfavorable histology.