PEComas are rare mesenchymal tumors that histologically present as a composite of epithelioid or spindle-shaped mesenchymal cells that immunohistochemically show a characteristic concomitant expression of melanocytic Human Melanin Black-45 (HMB45) and Melan-A and smooth muscle markers (actin and desmin) [1, 2]. Here, MLANA is linked to neoplasm with perivascular epithelioid cell differentiation.