Renal cell carcinoma arising from epithelial cells of the renal tubule is a highly aggressive and malignant tumor in all ages. Less than 2% of cases occur in childhood, relatively in older age group. Only a few pediatric series have been presented in the English literature. Tumor is presented with characteristic findings of flank pain, gross hematuria, and palpable mass. Although one half of the patients have metastasis at the time of diagnosis, most cases are currently being incidentally detected using improved imaging techniques. The overall prognosis in children appears to be similar to that in adults. Tumor stage and complete surgical resection have been reported as the most meaningful prognostic factors for the outcome. The incidence of metastatic disease is same as in adults. The effect of chemotherapy and immunotherapy, either preoperatively or postoperatively, is unclear. Cure is the most likely consequence in localized and completely resected tumors. Here, we present an 8-year-old boy with renal cell carcinoma demonstrating only hematuria without any pathological physical examination findings. The mass was described by abdominal ultrasonography and computed tomography in the left kidney. After the left nephroureterectomy, the patient was given no therapy.