Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: Common presentation of an unusual variation

Mullerian duct abnormalities are congenital malformations that are easily missed and can lead to incorrect diagnosis and unnecessary operative procedures. In this case, a young female presented with cyclic pelvic pain that continued after previous surgical resection of an ovarian cyst. Further investigation with clinical examinations and multimodality imaging demonstrated ipsilateral renal agenesis and a Class III Mullerian duct anomaly (MDA) requiring a second operative procedure. It is believed that this case is a variant of the described obstructed hemi-vagina with ipsilateral renal agenesis (OVIRA) anomaly as pathologically there was ipsilateral renal agenesis and complete vaginal agenesis in our case. It is imperative to have a high clinical suspicion of mullerian duct abnormalities when encountering a patient with other urogenital anomalies. This will decrease the amount of misdiagnoses, guide appropriate surgical intervention, and decrease the risk of future reproductive complications.