Therefore, considering morphology (large, plump cells with clear cytoplasm sometimes containing granular pigment Schmörl positive) and the immunohistochemical results (negativity for Cytokeratins, Vimentin, and α-SMA, and positivity only for HMB45) the diagnosis of Perivascular Epithelioid Cell tumour was considered the most plausible. This evidence concerns the gene PMEL and neoplasm with perivascular epithelioid cell differentiation.