OBJECTIVE In Wilms' tumour (WT), secondary malignancies caused by the side effects of intensive treatments remain one of the important problems. Therefore, there is a need for new studies to identify low- and high-risk groups for WT and to improve the treatment regimens of children in the low-risk group. In our study, we aimed to determine the prognostic significance of the cyclooxygenase-2 (COX-2) biomarker in WT. METHODS Our study included 24 patients diagnosed with WT between January 2010 and December 2019. The correlation between COX-2 expression and significant prognostic parameters was investigated by studying the COX-2 antibody using the immunohistochemical method. RESULTS COX-2 expression was observed in 22 of the patients and it was more evident in the epithelial component. No significant correlation was observed between COX-2 positivity and prognostic parameters. There was also no statistically significant difference between the two groups regarding survival (P = .563). CONCLUSIONS In our study, no significant relationship was found between significant prognostic parameters and COX-2 expression. Since COX-2 expression was observed in almost all patients, we consider that the COX-2 pathway is effective during the development phase of WT.