Obstructed hemivagina, ipsilateral renal agenesis (OHVIRA) syndrome, as well as didelphic uterus, is a rare, obstructed müllerian anomaly, affecting the physical, reproductive, sexual, and mental health of the patient. The diagnosis is often delayed because of unawareness of the fact that even common gynecological complaints like vaginal discharge can be associated with this rare entity. The most common symptoms at presentation, however, include pain in the abdomen and menstrual complaints. Therefore, while evaluating them, the possibility of this rare entity should be kept in mind; hence, emphasizing the importance of local examination. A 3D ultrasound and a magnetic resonance imaging are helpful in confirming the diagnosis. In this article, we report case series of OHVIRA syndrome with extreme clinical symptoms at presentation. We reviewed the literature and discussed various classifications used for this syndrome, including the newer classification based on the type of obstruction. The minimally invasive “vaginoscopic no-touch” technique of surgery is an alternative option available for adolescents where preservation of hymen seems to be important.