Background: Although Mullerian anomalies are relatively common they can be easily misdiagnosed as other gynecologic conditions leading to inappropriate treatment. Case: An 18-year-old woman presented to the hospital with abdominal pain and was found to have a 17-cm pelvic mass and absence of the cervix. Because of concern for recurrent endometrioma formation in the setting of a Mullerian anomaly, she underwent a hysterectomy. During surgery, she was noted to have complete uterine didelphys with cervical agenesis and a normal vagina. Summary and Conclusion: This extremely rare Mullerian anomaly represents one of the only descriptions to date of uterine didelphys with cervical agenesis and normal vaginal development. Appropriate identification and management of Mullerian anomalies is essential for guiding the care of these young, complex patients.
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