A 55-year-old woman presented with sudden right lower abdominal pain. Computed tomography demonstrated retroperitoneal hematoma associated with minus-density area, diagnosed as spontaneous rupture of angiomyolipoma. Super-selective transarterial embolization was performed, but anemia and right abdominal pain became worse in spite of conservative therapy including transfusion, indicating re-rupture of the renal tumor. Although partial nephrectomy was planned, right nephrectomy was finally performed because of massive intraoperative bleeding. Accurate diagnosis and prompt treatment are required when life-threatening rupture of renal tumor is suspected.