A 54-year-old male presented with massive hematuria with bladder tamponade. Computed tomography and magnetic resonance imaging showed a 7.5 x 6.5 x 4.5 cm. mass centered in the left renal pelvis, which is composed of macroscopic fat and soft-tissue component. Differential diagnosis included liposarcoma, lipoma and angiomyolipoma. Urothelial carcinoma was also undeniable because urine cytology was equivocal. Subsequently, a left nephroureterectomy was performed. Pathological examinations revealed a renal angiomyolipoma protruding into the renal pelvis. We present a rare case of angiomyolipoma in the renal pelvis and review the literature concerning these unusual findings of this common neoplasm.