Malignant epithelioid variant of angiomyolipoma has aggressive characteristics, against which conventional cytotoxic agents have been reported to be disappointingly inactive, and the prognosis of unresectable or recurrent disease is dismal poor. A 52-year-old man with a history of left nephrectomy for epithelioid angiomyolipoma was referred to our institution. The computed tomographic scan showed a soft tissue dense mass around the Rex's recess and behind the spleen, and a large pelvic mass. Specimens obtained by percutaneous needle biopsy confirmed the recurrence of malignant epithelioid angiomyolipoma. Everolimus was initiated at 10 mg per day for recurrent disease. Computed tomographic scans 2 months later showed the tumors to be markedly decreased in size. The patient has continued with this treatment on an outpatient basis without signs of disease progression over 7 months, as of February 2011. In this case, treatment with everolimus resulted in dramatic tumor response for the malignant epithelioid variant of angiomyolipoma.