子宫外苗勒氏腺肉瘤非常罕见,来源于卵巢、输卵管和腹膜间皮。文献中仅见13例卵巢和14例生殖道外的腺肉瘤的报道。本文报道1例卵巢腺肉瘤。患者32岁,日本妇女,G3P1,下腹疼痛7个月,无放射治疗史,盆腔检查发现右侧卵巢肿物并有子宫内膜异位囊肿,血清CA125 1 100 U/ml(正常为<35U/ml)。行右侧卵巢切除术。术中盆腹腔未见异常。术后10个月CT发现子宫直肠陷窝有肿瘤复发,血清CA125为950 U/ml。行全子宫、左侧附件、肿物、网膜及盆腔淋巴结切除术,术后4个月未发 Extrauterine Mullerian adenosarcoma is very rare, from the ovaries, fallopian tubes and peritoneal mesothelial. Only 13 cases of ovarian and 14 cases of genital sarcoma were reported in the literature. This article reports a case of ovarian adenosarcoma. Patients 32 years of age, Japanese women, G3P1, abdominal pain for 7 months, no history of radiation therapy, pelvic examination revealed ovarian masses on the right and endometriosis, serum CA125 1 100 U / ml (normal <35U / ml). Right ovariectomy. Intraoperative abdominal cavity no abnormalities. At 10 months after surgery, the tumor was found recurrent tumor in the rectum and the serum CA125 was 950 U / ml. Line of the whole uterus, left attachment, tumor, omentum and pelvic lymph node dissection, after 4 months without hair