Since HMB-45 immunoreactivity was described in renal AML and pulmonary CCST in 1991, the family of PEComas has expanded to include AML, LAM, CCST, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and clear-cell tumors of other anatomic sites in the past 2 decades [1,17-21]. The gene discussed is PMEL; the disease is neoplasm with perivascular epithelioid cell differentiation.