Angioleiomyoma (AL) is a benign tumor, composed of smooth muscle cells and thick-walled vessels. It occurs very rarely in female genital tract. The following is a case report of 45-year-old woman diagnosed with AL of the uterine with aberrant arterial supply from extra-gonadal vessels. Preoperative computed tomography examination revealed a large 30-cm tumor of the uterine, which was supplied by branches from the splenic artery. The woman was treated radically with hysterectomy and bilateral salpingo-oophorectomy. A review of the literature of AL diagnosed in the female genital tract was done.