Several case reports have provided most of the information available on perivascular epithelioid cell tumors, but with no consensus regarding diagnosis or treatment paradigms. Sunitinb is a VEGFR multitargeted tyrosine-kinase inhibitor that is regarded as a first-line treatment of clear cell-type metastatic renal-cell carcinoma after cytoreductive surgery. Our case was a 29-year-old male who presented with a metastatic left renal tumor and underwent left partial nephrectomy followed by adjuvant tyrosine kinase-inhibitor treatment. We report this case to present the peculiarity of this rare pathological variant, its clinical and diagnostic features, and challenges regarding treatment options, since the response of this rare tumor to adjuvant tyrosine kinase-inhibitor therapy has not been well described.