Objective: To investigate the clinicopathologic features of epithelioid angiomyolipoma (EAML). Methods: The morphological, immunohistochemical characteristics, clinical and follow-up data of 17 patients with EAML were retrospectively reviewed. Results: There were 5 males and 12 females of 17 cases, age ranged from 21 to 62 (average 31.2) years old, and 14 cases occurred in the kidney (3 cases with uterine leiomyoma and 2 cases with intrauterine pregnancy), 2 cases occurred in liver, 1 case occurred in abdominal cavity. The tumors maximum diameter was 3 to 15 cm. Microscopically, the tumor cells arranged in nests or sheets, cuff-shaped around the blood vessels. The cells were larger, ovoid or spindle, with abundant and eosinophilic cytoplasm, a big nucleus and obvious nucleoli, and a few mitotic figures. There were two cases with hemorrhage and necrosis of all. Immunohistochemistry showed that the tumor cells were positive for HMB-45, Melan-A and SMA. Vimentin, ER and PR positive rate respectively was 41% (7/17), 23% (4/17), 35% (6/17). All of them underwent radical surgery or surgical resection, and were followed up for 5–52 months after operation. Sixteen cases were alive, without recurrence or metastasis, while one case died of liver and lung metastases 2 years later. Conclusion: EAML mostly occurs in female, which may be associated with hormone levels. Most of them occur in kidney, then in liver and abdominal cavity. It has no specifications in the histopathologic features. Immunohistochemistry plays an important role in the differential diagnosis. Surgical treatment is the main method with a good prognosis, but there are possibility of recurrence or metastasis.