Summary
We report here in an angiomyolipoma with a prominent component of epithelioid smooth muscle cells seen in a 46 year-old female, measuring 24x14x10 cms and reaching to retroperitoneum having connection with left kidney. Microscopic examination revealed smooth muscle, adipose tissue, thick walled vessels and prominent epitheloid smooth muscle cells, in various proportions. İmmunohistochemical evaluation showed SMA positivity, Desmin positivity and HMB-45 positivity. We describe the case with literature findings and report it here in because of its potential for malignancy, its possibility of confusion with renal cell carcinoma and sarcomas.