Juxtaglomerular cell tumor (JGT) is a relatively rare, benign reninproducing tumor that causes hypertension, hyperaldosteronism, and hypokalemia due to excessive renin secretion. Here, we report a case of malignant JGT with pulmonary metastases. A 7-year-old male child patient was referred to the hospital for proteinuria found during a school medical checkup. An ultrasound examination revealed a tumor