Renal perivascular epithelioid cell tumor, which can be treated with surgery. Renal perivascular epithelioid cell tumor, also known as vascular ependymoma, is a very rare vascular tumor that most commonly grows in the lower extremities, pelvis, head and neck, and meninges, and tends to start as a painless mass, and most patients may experience persistent vague pain. Perivascular epithelioid cell tumor of the kidney is a rare soft tissue tumor, which is caused by long-term use of hormones or local trauma. It is usually classified into benign, borderline, and malignant types, and can be treated surgically, usually by excision of the lesion before metastasis occurs, or by radical resection. It is recommended to go to regular hospitals for timely diagnosis and treatment of renal perivascular epithelioid cell tumors, so as to strive for a speedy recovery.