Objective: Tuberous sclerosis (TS) is a genetic disorder affecting cellular differentiation and proliferation, which results in hamartoma formation in many organs like skin, brain, lung, kidney and heart. Method: A seventeen-year-old female with history of facial angiofibromas a 10 years before, right renal tumor diagnosed a year before as theratoid-rhabdoid tumor, with chemoradiation as adjuvant treatment, cortical tubers by neuroimaging a few months before, and diagnosis of lung lymphangioleiomyomatosis recently. After last diagnostics, we reviewed again the renal tumor, new orientation of tissue, with new histological sections were performed. (Fig) Results: At histological examination, the kidney showed an infiltrating tumor, very cellular, consisting mainly of polygonal cells with eosinophilic cytoplasm, other multinucleated similar to the ganglion cells, also small hamartomatous areas with smooth muscle, fat and blood vessels proliferation. The tumor cells expressed HMB45, MART-1, SMA, VIM and CD10, CKAE1/3 were negative. (Fig)  Conclusion: The majority of renal angiomyolipomas is sporadic and 50 to 80 % occurs as part of TS, and their partnership is more close with epithelioid variant, recently, a rare entity with aggressive behavior, difficult histological characterization and poor prognosis.