Cyclooxygenase 2 has become an important pharmacological target in anticancer therapy due to the over expression of COX 2 in pathological conditions. Wilms’ tumor is a common kidney cancer in children which has shown an increase in COX 2 enzyme level. Here we reviewed various articles that considered the cyclooxygenase 2 changes specifically in Wilms’ tumor regarding the mechanisms of action and inhibitors of COX 2.