A 64-year-old man with flank pain was diagnosed with right renal angiomyolipoma (AML) with tumor thrombus invading the inferior vena cava (IVC). Computed tomography showed a 55 mm IVC tumor thrombus with fat density. The patient underwent radical nephrectomy and IVC thrombcetomy with uneventful postoperative recovery. Pathological diagnosis was AML without malignancy. No recurrence has been observed for 18 months after surgery. We reviewed 60 case reports of AML with venous involvement. Furthermore, we discussed differential diagnosis between AML and other renal tumors mimicking AML with caval involvement.