Summary

PEComas (perivascular epithelioid cell tumours) are rare mesenchymal tumours, characterised by epithelioid, clear cell morphology with coexpression of melanocytic and smooth muscle markers. They are usually seen in adult females in a variety of anatomical locations, of which, kidney, lung and the gynaecologic tract are the most common. We present a case of PEComa arising from the kidney of a 12-year-old boy.