OBJECTIVE To present a new case of an uncommon complication of a benign renal tumor. To our knowledge, there are only few published cases of benign renal angiomyolipoma (AML) presenting with tumor thrombus in females. Epithelioid angiomyolipoma is a recently described rare variant of renal angiomyolipoma. It can occur in patients with or without tuberous sclerosis, and may potentially be malignant. Benign renal angiomyolipoma AML rarely presents with evidence of extension into the renal vein, inferior vena cava (IVC) or atrium. We report a case of a benign renal AML with a tumor thrombus to the IVC in a 46-year-old female who presented with right-sided flank pain associated with a right sided abdominal mass. METHODS Right Radical nephrectomy with IVC tumor thrombectomy. RESULTS Patient is totally asymptomatic. At 1 month after surgery, an abdominal ultrasound showed no evidence of thrombus within the IVC. CT scan of the abdomen at 3 months post-operatively showed no evidence of recurrence. CONCLUSIONS Surgical treatment of angiomyolipoma with IVC thrombus is warranted in view of risk of malignancy and to prevent tumor embolus to the heart or lungs.