Angiomyolipoma is the most frequent neoplasm of the kidney. It may have a wide range of clinical manifestations, but it is usually detected incidentally on cross-sectional images. Rupture and hemorrhage of angiomyolipoma is an important concern for large lesions. We present the case of a 42-year-old female, with no history of urinary stones, who presented with a sudden-onset left flank pain for eight hours. Upon examination, she was tachycardic and hypotensive. Abdominal examination revealed a left-sided flank mass. Abdominal computed tomography (CT) scan with intravenous contrast demonstrated a heterogeneous mass lesion in the left kidney with mixed attenuation and had a macroscopic fat density that was surrounded by a large hematoma. Subsequently, transcatheter renal artery embolization was performed with no complications. The procedure was able to control the active bleeding. Then, a total nephrectomy was performed, and the hematoma was evacuated during laparotomy. Spontaneous nontraumatic renal hemorrhage is a very rare condition. Clinicians should keep a high index of suspicion for this condition when they encounter a patient with the clinical triad of flank pain, flank mass, and hypotension. The case demonstrated the role of endovascular embolization of angiomyolipoma to control life-threatening hemorrhage.