Angiomyolipoma (AML) is a mesenchymal neoplasm composed of a variable mixture of adipose tissue, smooth muscle cells, and anomalous blood vessels. In the uterus, only a few cases have been reported. We described a uterine AML without evidence of tuberous sclerosus (TS). The clinical presentations of uterine angiomyomatous lesions are nonspecific. The diagnosis of angiomyolipoma can be established with ultrasound and/or computed tomography (CT). The patients whose lower abdomen mass in the abdominopelvic ultrasound and dysfunctional uterine bleeding should be considered AML in the differential diagnosis.