The World Health Organization defines perivascular epithelioid cell tumors (PEComas) as mesenchymal tumors and tumor-like conditions composed of epithelioid cells with a perivascular distribution. These tumors may show benign or malignant histology and/or biological behavior. However, the pathological features of malignancy may not correlate with biologic aggressiveness, and the criteria for malignancy are not clearly defined. Abdominopelvic PEComas are very rare and have been reported in various locations, including kidney, liver, pancreas, gastrointestinal tract, genitourinary tract, peritoneum, and retroperitoneum. Cross-sectional imaging techniques, including computed tomography (CT) may play an important role in the accurate detection and characterization of these tumors. We present the third case of an extremely rare PEComa with perirenal location, discuss CT findings and differential diagnosis.