Wilms tumor, also called nephroblastoma, is a renal neoplasm with the highest incidence in early childhood and rarely affects adults. The objective of the present study was to report a case of a child who was referred to a private clinic in the city of Petrolina - Pe to undergo an imaging exam and review the literature regarding Wilms tumor. It was developed through a case report, together with literature findings in the Pubmed database. In the report, the patient had a radiological diagnosis of Wilms tumor, indicating chemotherapy treatment and subsequent surgical intervention. In the end, it was found that the diagnosis must be made by correlating radiological and surgical findings and with the anatomopathological study.