Background: Robert's uterus, a rare congenital abnormality of Mullerian duct development, has oblique septum and non-communicating asymmetric hemi-cavity. Key clinical characterization of the congenital disorder is presence of hematometra and severe dysmenorrhea, some patients experience acute abdominal pain in association with menstruation. Due to the difficulty of preoperative diagnosis and high rate of misdiagnosis, Preoperative assessment matters the choice of emergency surgery and avoid a second surgery. No systematic literature review in detail has been reported previously.Case presentation: We reported a rare case of Robert’s uterus with severe abdominal pain during menstruation, in which the patient initially underwent emergency laparoscopic right ovarian cystectomy, right salpingectomy, and pelvic adhesiolysis on suspicion of ovarian teratoma torsion. However, dysmenorrhea still existed or even aggravated after the operation, hysteroscopic surgery was performed three month later, which revealed two asymmetric uterine cavities, hematometra was located in the right-side blind cavity, and thus diagnosed as Robert’s uterus and severe uterine adhesion, which, to our knowledge, has not been reported previously. Hysteroscopic incision of the septum and intrauterine adhesion were performed. Subsequently, Two-year follow-up showed no obvious dysmenorrhea recurrence. Conclusion: Robert's uterus is uncommon but can mimic other common and acute abdominal disease. Advance imaging technologies, such as three-dimensional ultrasound and magnetic resonance imaging combined with hysteroscopy and laparoscopy are less invasive for diagnosis and treatment of Robert’s uterus. Septal resection is main surgical procedure, combining laparoscopy and hysteroscopy is conducive and less invasive treatment.