Wilms tumour (WT) is one of the most common paediatric renal malignancies. While the overall survival (OS) has improved above 90% in high-income countries, the outcomes remain inferior in low- and middle-income countries (LMICs) due to the healthcare system and socioeconomic barriers. This study aimed to document the demographic profile, clinical features and treatment patterns, and to evaluate the survival outcomes of children with WT managed in our institute. We retrospectively analysed patients diagnosed as WT between January 2000 and December 2023 at the Paediatric Oncology Unit of a tertiary care cancer centre in Southern India. Descriptive statistics were used to summarise the demographic profile, clinical features and treatment patterns. Event-free survival (EFS) and OS were estimated using Kaplan–Meier methods. Sixty-nine children were diagnosed with WT at our centre during the study period. The median age at diagnosis was 34 months (range: 8–159 months), with a slight male predominance (M:F ratio 1.22:1). Seventeen (25%) children without any image-defined high-risk factors underwent upfront nephrectomy while the remaining 52 (75%) children received preoperative chemotherapy followed by delayed nephrectomy. The stage distribution was as follows: Stage I–24 (35%); Stage II–15 (22%); Stage III–13 (19%); Stage 4–13 (19%); Stage 5–3 (3.5%); and Unknown–1 (1.5%). With a median follow-up of 68.5 months, the 5-year EFS and OS rates of our study cohort were 69% (95% confidence interval (CI): 56%–78%) and 78% (95% CI: 65%–87%), respectively. The most common causes of treatment failure were relapse (22%) and treatment abandonment (11%). Multivariate analysis revealed that advanced stage and upfront nephrectomy were independent predictors of inferior survival. Survival outcomes for WT in LMICs remain inferior despite multidisciplinary management. A hybrid approach – tailoring surgical timing to individualised risk – appears pragmatic. Bridging the survival gap requires addressing socioeconomic barriers and strengthening multidisciplinary care.