Perivascular epithelioid cell tumor (PEComa) is a less common mesenchymal tumor, expressing melanocytic and myogenic markers such as actin, desmin, calponin, human melanin black (HMB) 45, melanA, and microphthalmia-associated transcription factor (MITF) [4]. The gene discussed is DES; the disease is neoplasm with perivascular epithelioid cell differentiation.