Renal angiomyolipoma (AML) is a relatively rare tumor that is generally considered as merely benign. However, epithelioid AML (EAML), an uncommon subtype, is associated with potentially malignant behavior. We herein present the case of a 60-year old male patient who had undergone left nephrectomy with left adrenalectomy and lymphadenectomy for a renal tumor 12 years earlier, and presented to our hospital with dull abdominal pain. The histology report after the previous surgery had revealed an AML of the left kidney with a maximal diameter of 17 cm. Imaging studies demonstrated a large tumor of 13 cm in diameter in the area of the resected kidney, as well as hepatic and peritoneal metastases. Computed tomography-guided core needle biopsy of the mass and revision of the histology of the nephrectomy revealed an EAML. Four years after a two-stage resection of the recurrences the patient is in excellent condition and free of disease. From this case report and the literature review on EAML, it appears that correct histological diagnosis of this subtype of renal AML is crucial. Erroneous diagnosis of simple renal AML instead of EAML may lead to insufficient postoperative management. Clinicians should be aware of the malignant potential of EAML and the need for long-term follow-up. As effective surgical and emerging medical treatment options are available, timely detection of recurrent disease may lead to improved outcome.