Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors arising from histologically and immunohistochemically distinctive perivascular epithelioid cells that express both myogenic and melanocytic markers such as HMB 45, HMSA-1, Melan A/Mart 1, microophtalmia transcription factor (Mitf), actin, and less commonly desmin [1, 2]. The gene discussed is DES; the disease is mesenchymal cell neoplasm.