Renal Angiomyolipoma (AML) is a rare benign tumor As it is usually asymptomatic and small, AML sometimes may cause acute abdomen by spontaneous rupture and hemorrhage that may be life threatening in some cases for which surgical management is necessary. A 58-year-old female patient was admitted for right side and right upper abdominal pain, nausea and vomiting. Right upper abdominal and right side sensitivity were remarkable on physical examination. Whole blood count revealed the ongoing with steady remarkable decrement in hematocrite and hemoglobin. Radiological examination showed right kidney mass with retroperitoneal hematoma. Patient underwent a right nephrectomy with mass excision. Her postoperative period was uneventful. On this case report we conclude that; angiomyolipoma may cause serious complications by the spontaneous rupture and life threatening hemorrhage. In case of massive hemorrhage and/or whole renal involvement, nephrectomy is the most feasible surgical treatment of all the other treatment methods.