Abstract: ObjectiveTo investigate the clinical and pathological characterisitics and treatment conditions of angiomyolipomas occur in multiple parts of the body and establish a reasonable standard to provide some guide for clinical diagnosis and treatment. Methods Clinical-pathological features, concidence between preoperative image diagnosis and postoperative diagnosis,clinical treatments and follow-up results of 156 AMLs from 1981 to 2010 were retrospectively analyzed . ResultsThe two common involved organs were kidney (77%, 120/156) and liver (14 %, 22/156). 52% (81/156) of renal and liver AMLs were diagnosed as malignancy by image diagnosis, and 74 of them were treated by radical nephrectomy, liver interventional therapy or lobectomy of liver. Follow-up results in 124 cases, only one (0.8%, 1/124) renal epithelioid AML was found liver metastasis 1 year after nephrectomy.No recurrence or metastasis was found in all other cases within a mean follow-up period of 48 months. ConclusionIn this sizeable single institutional AML series, the kidney and liver were the first two sites of involvement, and most of them presented a benign clinical course,although few EAMLs were malignant. To avoid overtreatment of benign AMLs, pre- or intraoperative pathological diagnosis was essential.