Wilms' tumor, nephroblastoma is the most common renal tumor in children. Treatment outcomes have improved dramatically over the past few decades due to applying of multimodality therapy consisting of surgery, radiotherapy and combination chemotherapy. There are two representative protocols produced by SIOP (Internationl society of Paediatric Oncology) and COG (Children Oncology Group) which is previously NWTS (Nationlal Wilms' tumor study) group in treatment of Wilms' tumor. The current protocol used by the COG undergoes upfront surgery prior to initiation treatment of chemotherapy. But, the protocol used by the International Society of Paediatric Oncology (SIOP) performs staging after preoperative chemotherapy and then surgery and postoperative chemotherapy. Current protocols becomes conducting to incorporate biologic features to stratify patients for therapy. Treatment strategies focuses on minimzing late effects of treatment while producing an excellent survival. This review presents a summary of these advances and outline the current molecular markers, pahtoloic and radiologic diagnostic method and treatment of Wilms' tumor.