Background. Nephroblastoma is the commonest renal tumour seen in children. It has a good prognosis in developed countries with survival rates estimated to be between 80% and 90%, while in Africa it remains low. Method. Retrospective study of patients diagnosed with nephroblastoma who are seen at 4 paediatric oncology units, representing 58.5% of all South African children with nephroblastoma and treated following SIOP protocol between January 2000 and December 2010. Results. A total of 416 patients were seen at the 4 units. Over 80% of our patients were African and almost 10% of mixed ethnicity. The most common stage was stage 4. The median survival was 28 months after diagnosis with the mixed ethnicity patients recording the longest duration (39 months) and the white patients had the shortest median survival. The overall 5-year survival rate was estimated to be 66%. Stage 2 patients did significantly better (85%). Conclusions. Our patients are similar with regard to gender ratio, median age, and age distribution as described in the literature, but in South Africa the more advanced stage disease seen than in other developed countries is translated into low overall survival rate.