PEComa ( Pure Epithelioid Angiomyolipoma ) of kidney

A 60-year-old male patient presented with complaints of loss of appetite and bloated abdomen of two weeks duration. The radiological examination revealed large mass involving left kidney. With a clinical and radiological diagnosis of renal cell carcinoma, radical nephrectomy was done. Histopathological examination revealed a neoplasm composed purely of epithelioid cells. Based on histopathological and immunohistochemical findings a diagnosis of Epithelioid Angiomyolipoma of kidney was made. We are reporting this case because of the rarity of this variant of angiomyolipoma and to highlight the diagnostic difficulties of this entity when compared to the more common classic angiomyolipoma.