Advancements in surgery, radiotherapy, and chemotherapy have greatly improved the cures rates for children with Wilms tumor. However, a number of therapy-related late effects have been observed in long-term survivors. Generally, late complications are a consequence of treatment type and intensity; the use of radiotherapy and anthracyclines increases the risk of these complications. This commentary highlights some clinically significant late sequelae-musculoskeletal effects, cardiac toxicity, reproductive problems, renal dysfunction, and the development of second malignant neoplasms. Careful medical and epidemiological monitoring of survivors can provide critical information to further optimize therapy while minimizing long-term sequelae.