A 26-year-old man presented with acute renal insufficiency, and severe lower extremity swelling. Computed tomographic scan revealed retroperitoneal lymphadenopathy encasing both ureters and the inferior vena cava. He underwent placement of ureteral stents to relieve the obstruction and afterward underwent lymph node biopsy, which revealed Kaposi's sarcoma. He subsequently was diagnosed with acquired immunodeficiency syndrome. Abdominal and lower extremity venous duplex ultrasound did not show any evidence of deep vein thrombosis. The inferior vena cava measured 3.5 mm in diameter and was encased by retroperitoneal lymphadenopathy. Bilateral transfemoral venography and intravascular ultrasound demonstrated significant compression of the inferior vena cava below the renal veins. Endovascular treatment was followed with primary stenting under intravascular ultrasound guidance. His symptoms improved with reduction in swelling. At 1-year follow-up, the patient was ambulatory with mild symptoms, and on venography the iliac vein and inferior vena cava stents were widely patent.