Uterine malformations occur early in embryogenesis due to abnormalities in development of paramesonephric (Mullerian) ducts. Mullerian duct anomalies can range from complete agenesis of the uterus to nonpathological variants (e.g., mildly arcuate uterus). This case study discusses an example of Herlyn-Werner-Wunderlich syndrome in an adolescent female with multiple congenital anomalies who presented with chronic, worsening abdominal pain and an enlarging mass in her lower left abdomen and pelvis. This case demonstrates the importance of a complete history and physical examination, appropriate imaging modalities, and an interdisciplinary approach to appropriately treat this patient.
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