Objective: To investigate the imaging features and clinicopathological features of perivascular epithelioid cell tumor (PEComa) of the genitourinary system, and to improve the understanding of PEComa. Methods: The clinical, pathological and imaging data of 5 patients with PEComa confirmed by surgical pathology were retrospectively analyzed. Results: The age of the five patients ranged from 9 to 63 years, with a mean age of 40.8 years Among the 5 patients, 4 lesions were located in the kidney and 1 lesion in the vagina. The diameter of the lesion is 0.9~19 cm. Postoperative gross specimens showed that section of the tumor was gray and gry-red, with bleeding, toughness and envelope. Microscopically, the tumor was epithelioid and fusiform, with abundant cytoplasm, eosinophilic, granular, vacular, obvious nucleolus, few mitotic images, and large necrosis. CT plain scan and enhanced examination and MRI plain scan and enhanced examination were performed in 2 cases, only CT plain scan and enhanced examination in 3 cases. Homogeneous enhancement was observed in 2 cases, and uneven and obvious enhancement was observed in 3 cases of larger lesions, with liquefaction necrotic area. There are large vascular shadows in or around the lesion. Immunohistochemistry showed positive expression of HMB45, and partial positive expression of MELAN-A, SMA CD-31, CD34 and S-100. Conclusions: The imaging findings of perivascular epithelioid cell tumor of genitourinary system have certain characteristics, The CT image density is uniform and the enhancement is uniform and obvious, and the enhancement degree is lower than with of normal renal parenchyma. MRI image signal is uniform, the enhancement is uniform and obvious. When the lesion is large, the CT image density is uneven, liquefaction necrotic area may appear, the enhancement scan shows uneven enhancement, the solid part of the enhancement is obvious, and the blood supply of large vessels is visible, and the internal necrotic area does not show obvious enhancement.