1病例报告患者女,14岁。因无月经来潮来院就诊。否认周期性下腹疼痛、膀胱刺激及漏尿等病史。妇科检查:乳房虽已开始发育但较小,乳头不明显;阴毛大致呈女性分布,较稀少;大小阴唇发育好,尿道口正常,阴道前庭口有处女膜。染色体检查:46,XX。超声检查:盆腔内反复扫查,未见子宫、输卵管和卵巢,但有阴道。最后诊断:先天性无子宫及附件。 1 case report patient female, 14 years old. Because no menstrual cramps to hospital. Denied periodic abdominal pain, bladder irritation and leakage of urine and other medical history. Gynecological examination: although the breast has begun to develop but smaller, nipple is not obvious; pubic hair roughly female distribution, more rare; size labia good, normal urethral orifice, vaginal vestibule hymen. Chromosome examination: 46, XX. Ultrasonography: pelvic repeated scanning, no uterus, fallopian tubes and ovaries, but there is vaginal. The final diagnosis: Congenital absence of uterus and attachment.