卵巢表皮样囊肿是属于单胚层高度特殊性分化的卵巢成熟囊性畸胎瘤（皮样囊肿）的一种。本瘤尚未列入世界卫生组织国际卵巢肿瘤组织分类中。近二十年国内外文献报告仅２２例，较罕见，而同时伴有先天性子宫缺如的病例尚未见报道。我院目前发现一例，现报道如下。患者，女，７岁 Ovarian epidermoid cyst is a kind of ovarian mature cystic teratoma (dermoid cyst) which belongs to the highly specific differentiation of single embryo. This tumor has not yet been included in the World Health Organization International Ovarian Tumor Classification. Nearly two decades of domestic and foreign literature reports only 22 cases, rare, but accompanied by congenital uterine absence of cases have not been reported. Our hospital now found a case, are reported below. Patient, female, 7 years old