To the Editor, Perivascular epithelioid cell tumors (PEComa) are a collection of uncommon mesenchymal tissue tumors made up of perivascular epithelioid cells. Due to the low incidence of the disease and the lack of characteristic manifestations, it is easy to be missed and misdiagnosed clinically. We will present a case of malignant ureteral PEComas and discuss their clinical, radiological, and pathologic characteristics in light of pertinent literature.