Pedunculated submucosal leiomyomas of the uterus that prolapse into the vagina are common. In extremely rare cases, large pedunculated submucosal leiomyomas may lead to bilateral obstructive uropathy, causing severe renal dysfunction and potentially being misdiagnosed as intracervical leiomyoma. The present study describes the surgical treatment of a patient with a large prolapsed pedunculated submucosal uterine leiomyoma, which was misdiagnosed as an intracervical fibroid. The patient, of menopausal age, presented with uterine bleeding, anemia and severe renal dysfunction. Upon a physical examination, suspicion arose for a cervical leiomyoma, prompting the decision for imaging. Both transvaginal ultrasound and computed tomography, as well as magnetic resonance imaging confirmed the diagnosis of intracervical leiomyoma, accompanied by bilateral obstructive uropathy due to ureteral compression. The surgical management of the patient with laparotomy was decided. Intraoperatively, a large pedunculated submucosal uterine leiomyoma prolapsing into the vagina was identified. Total hysterectomy and bilateral salpingectomy-oophorectomy were performed. The immediate post-operative course was uneventful. At 6 months following surgery, the complete recovery of renal morphology and function was observed. The patient continues to undergo regular follow-up assessment to date. In the present study, a brief literature review is also provided, emphasizing the significant diagnostic and surgical challenges that may arise in the management of patients with large pedunculated submucosal uterine leiomyomas prolapsing into the vagina.