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Angiomyolipoma (AML) is a well-known rare soft tissue tumor involving the kidneys, liverand other organs. Epithelioid angiomyolipoma is a recently recognized variant ofangiomyolipoma, which is characterized by the presence of polygonal cells with denselyeosinophilic cytoplasm and varying degrees of nuclear atypia. Immunreactivity with HMB-45and CD68 are helpful for its identification. We report a case of epithelioid angiomyolipoma ofthe kidney that occurred in a 19-year-old woman.