Nephroblastoma, though uncommon, is an important neoplasm because when present, it is usually found in young patients. Neoplasia is commonly a differential diagnosis in geriatric patients and infrequently considered in patients younger than four years of age. There are few neoplasms that affect young patients, nephroblastoma being one; other examples include lymphoma, which has a bimodal age distribution, and histiocytic sarcoma. Nephroblastoma is classified as a primitive neuroectodermal tumor (PNET), which arises from embryologic tissue. It is because of the tumor’s origin in embryologic tissue that this neoplastic process affects young animals. Consequently, when presented with a young patient affected by an unknown neoplastic process in the kidney or spinal cord, it is important to place nephroblastroma on the differential diagnosis list.