Wilms tumor is a rare type of cancer that mainly affects children. It’s the most common cancer of the kidneys in children. Although it is usually seen under the age of 3, it can also be diagnosed at older ages. Extrarenal Wilms tumor (ERWT) is very rare, comprising <1% of all Wilms tumor cases. It is most commonly seen in the retroperitoneum and pelvic region and has been reported in a limited number of patients in the literature. This report presents a patient with ERWT of the cervix who underwent FDG PET/CT imaging.