Malignant renal epithelioid angiomyolipoma (EAML) is an extremely rare disease with a poor prognosis, and currently there are no uniform criteria for its biological behavior. Here, we present a case of malignant renal EAML with TFE3 gene amplification in a 53-year-old woman. Four months after surgery, unenhanced computed tomography scans showed recurrence as well as metastasis in the abdomen and lung. The patient succumbed to rapid neoplastic progression of the disease 6 months later.