直肠阴道隔清亮细胞腺癌是非常罕见的疾病。我们报道了一例55岁女性患者，在2017年子宫全切术和双侧输卵管卵巢切除术后被诊断出阴道直肠隔区块状物，因阴道出血住院就诊。盆腔磁共振成像显示阴道直肠区囊性实性肿块大小约为110毫米×100毫米×140毫米。术后病理诊断为清亮细胞腺癌。腹腔剖腹手术显示阴道直肠隔为坚固的肿块。手术切除肿瘤后，患者接受了8个周期的综合化疗，治疗残留病变，并达到完全缓解。由于其生长模式罕见，细胞形态和免疫表型不具特异性，临床和病理诊断难度大。通过介绍此例的诊断和治疗，并回顾文献，为临床医生识别、诊断和治疗这种罕见疾病提供了相关参考。版权所有 © 2023作者。由 Wolters Kluwer Health, Inc. 发布。

Primary clear cell adenocarcinoma of the rectovaginal septum is a very rare event.We reported a case of a 55-year-old woman diagnosed with a lump in the vaginal rectal septum after undergoing hysterectomy with bilateral salpingo-oophorectomy in 2017, who was admitted to our department due to vaginal bleeding. Magnetic resonance imaging of the pelvis indicated the vaginal rectal space cystic and solid mass about 110 mm × 100 mm × 140 mm in size.The pathological diagnosis of postoperative was clear cell adenocarcinoma.Abdominal laparotomy showed a solid block of the vaginal rectal septum. Surgery was performed to reduce the tumor.This patient received 8 courses of combined chemotherapy courses after surgery for the residual lesion and achieved a complete response.Due to the rare observation of the growth pattern, the cell morphology and immune phenotype are not specific, and clinical and pathological diagnosis is difficult. Introducing the diagnosis and treatment of this case and reviewing the literature provide a relevant reference for clinicians identification and diagnosis and treatment of this rare case.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.