Perivascular epithelioid cell tumour (PEComa) is a rare mesenchymal tumour made up of clear perivascular cells with epithelioid characteristics, which co-expresses muscle and melanocytic markers with a component of spindle cells, like sarcoma and variety of other tissues. This time, we present the case of a young patient with a tumour in the dorsal region of progressive growth, compatible with PEComa of soft tissue after histopathological and immunohistochemical analysis.