The triad of uterine didelphys, obstructed hemivagina and ipsilateral renal anomaly known as OHVIRA syndrome, formerly known as Herlyn-Werner-Wunderlich syndrome, is a rare congenital urogenital malformation. It represents a diagnostic dilemma because of the regular menstruation and nonspecific abdominal pain. We present the case of a 13 year old pubertal girl presenting with severe dysmenorrhea. After evaluation with history and investigations she was diagnosed to have bicornuate uterus with hematometrocolpos, left sided hematosalpinx and left renal agenesis. After laparotomy she was diagnosed to have uterine didelphys with left sided hematometra, hematocolpos and ahematosalpinx due to left sided obstructed hemivagina and left renal agenesis (OHVIRA Syndrome). Surgery was done by abdominoperineal route. She had drainage of hematometra, hematocolpos and hematosalpinx and repair of obstructed hemivagina through perineal route. In conclusion, awareness of such anomaly is a prerequisite to early and prompt diagnosis. Surgery should not to be delayed after diagnosis of OHVIRA syndrome, so that complications can be avoided and reproductive function can be preserved.