Perivascular epithelioid cell tumors (PEComa) are a rare type of mesenchymal tumor arising from perivascular epithelial cells. The prognostic classification of PEComas, the nature of their biological behaviour and the criteria determining their malignancy potential remain uncertain. PEComas usually show immunoreactivity for melanocytic (HMB-45 and/or melan-A) and smooth muscle (actin and/or desmin) markers. Surgery is the most recommended primary treatment. We reported an uterin PEComa with atypical immunohistochemical features.