Angiomyolipomas (AML) are described as benign renal tumors which consist of vascular, smooth muscle, and adipose tissue components. Abnormal differentiation of renal mesenchyme is the main reason. It forms only 2-6,4% of the whole renal tumors, but it is the most common among the benign tumors. Most of them are asymptomatic and we can see it in almost 13 of 10.000 adults. Eighty percent of the AML is sporadic and 20% of them can be seen as a part of tuberous sclerosis complex (TSC). Epithelioid AML (EAML) is a rare subtype that can have malign or benign progression, it can be misdiagnosed as Renal Cell Carcinoma (RCC) and it is hard to differentiate them. EAML have some common diagnostic characteristics in presentations with other renal tumors. Because of that, we need to make an immunohistochemical examination to differentiate it from RCC, especially. We presented a 34 years old female patient with the pathologic result of the EAMLin the partial nephrectomy material.