Angiomyolipoma usually present as incidental findings on routine imaging or laparotomy, but rarely they may give rise to massive hemorrhage. If bleeding occurs, the treatment of choice is to save life either by angiography with selective embolisation or surgery. Herein, we reported a case of a 32-year-old man who came to our hospital complaining of sudden colicky pain in the right hypochondria region, associated with nausea and vomiting. On physical examination, the patient was in a state of shock; blood pressure of 60/36 mmHg, heart rate of 160 beats/min, high abdominal distention, and non-coagulated blood was extracted from abdominal puncture. The abdominal computed tomography (CT) and arteriography showed a 20× 15 × 15 cm mass suggestive of spontaneous rupture of liver neoplasm and intraperitoneal hemorrhage. On laparotomy, 1500 mL of non-coagulated blood was found, and the tumor located at right upper abdomen, which originated from retroperitoneum.The tumor was resected totally with hematoma and sutured the bleeding vessels. The histological study of the resected mass revealed the presence of angiomyolipoma.