Angiomyolipoma (AML) is a frequent benign renal tumor, corresponding to 0.3-3% of all renal masses. Eighty percent of AML occur sporadically; however, 10% have a hereditary component. We describe the case of a 41-year-old female patient with left abdominal pain associated with emesis, diarrheal stools, and dysuria. Upon admission, a computed tomography scan showed a left renal mass with tumor thrombus extending into the left renal vein and inferior vena cava (IVC). The patient underwent an open left radical nephrectomy plus thrombectomy. At 6months of follow-up, the patient is without tumor recurrence and asymptomatic.