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Wilms tumor is the second most common intraabdominal malignancy in childhood. Vessel invasion with thrombus formation is a well-known phenomenon in Wilms tumor and is found in 30% of these children. This report describes a 4-year-old boy with a left kidney Wilms tumor and extensive thrombi formation with intracardiac extension. He underwent a left radical nephrectomy under cardiopulmonary bypass following a single dose of chemotherapy. Emergency surgery with cardiopulmonary bypass may be lifesaving in children with cardiac compromise in the presence of extensive thrombosis.