Uterine malformations results from the defective fusion of Mullerian ducts during the development of the female genital tract. The least common form of this malformation is OHVIRA syndrome, a very rare congenital anomaly of the urogenital tract involving both Müllerian ducts and Wolffian structures. It is characterized by a triad of uterine didelphys, ipsilateral renal agenesis and obstructed hemivagina. There is a wide variety of phenotypic presentations recognized as a spectrum of disease rather than a separate entity. It usually presents after menarche but may have delayed presentation depending upon the type.
Case report: A multiparous woman with previous two cesarean deliveries presented with complaints of pelvic pain, urinary retention and hematuria and an ultrasound report suggestive of bicornuate uterus with large hematocolpos /hematometra. On Examination under anesthesia –a huge cystic bulge on the anterior vaginal wall was seen. On MRI, bicornuate bicollis uterine anatomy was seen with right hematotrachelos. IVP was suggestive of absent right kidney. Laparotomy with the Right hemihysterectomy along with drainage of hematocolpos was done.