We present a case of bicornuate uterine horns with complete cervico-vaginal agenesis and associated congenital uterovaginal fistula. The patient presented with cyclical hematuria, amenorrhea, and abdominal lump. The vagina was blind-ending. The cystoscopic examination during cyclical hematuria revealed bloody efflux through a small fistula on the posterior wall of the urinary bladder. The magnetic resonance imaging (MRI) showed bicornuate uterus and cervical agenesis. Hysterectomy and repair of the uterovesical fistula was done. The vagina was reconstructed using an amniotic mould. The report underlies the importance of MRI in diagnosing complexity of such rare anomalies. It also stresses for the need of hysterectomy if cervicovaginal agenesis is present.
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