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Introduction: Nephroblastoma is the most common type of malignant renal tumor in children, but hemorrhage in ruptured nephroblastoma is rare. Case presentation: A three-month-old boy was admitted to our hospital with a large right renal tumor and hemorrhagic shock. Laparotomy showed that the tumor had invaded the right lobe of the liver; therefore, surgeons decided to perform only hemostasis and a tumor biopsy. The following day, intratumoral bleeding persisted, necessitating continuous blood infusion, resulting in abdominal compartment syndrome. Therefore, tumor resection was performed. Intra-tumoral hemorrhage continued during the operation, resulting in bradycardia and chest compressions. Histopathological examination confirmed a diagnosis of nephroblastoma. The postoperative course was uneventful. Conclusion: This report underlines the need to choose treatment based on both extratumoral and intratumoral bleeding, keeping in mind the risks of each treatment option.