Background In contrast to all other known kinds of regulated cell death, cuproptosis is a unique copper-dependent cell death pathway that has a close connection to mitochondria and metabolism. The pathogenesis of Wilms tumor (WT), a common pediatric abdominal tumor, has yet to be fully defined. Besides, previous studies on lncRNAs related to Cuproptosis in WT and the connection between Wilms tumor, the microenvironment, and cuproptosis are still scarce.Results Our results show that Individuals with WT and high-risk group identified utilizing the signature were related to poorer survival outcomes. The study revealed that high-risk group had a worse prognosis (P < 0.001) in contrast with the group of low-risk. The risk score AUC for this signature was 0.818, which was higher than the risk score for age (0.524), sex (0.580), race (0.489), stage (0.673) and the modeling of ferroptosis-related lncRNAs (0.775). Individuals in the group of low-risk had an elevated TIDE scores ratio and were more sensitive to 13 drugs, including BI-2536, EX-527, IspinesibMesylate, KIN001-135, etc.Conclusions Our model can precisely predict the WT patients' prognosis and differentiate between low- and high-risk WT patients. It is hoped that this study will give a novel method for clinical prognosis and appropriate WT patients therapy.