Introduction: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, is a rare Mullerian duct anomaly with uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. In most cases, Patients who rarely present this structural anomaly, start to experience sympotms such as chronic pelvic pain and mass sensation that usually swap that star wirh menarche and variate with the menstrual cycle. Objective: this is an original article which aims to describe the wide range of symptoms presented by patients with (OHVIRA) syndrome, how their quality of life is affected due to underdiagnosis, and how 2D and 3D ultrasound is an important tool for the diagnosis of an adequate surgical technique. Matherials and methods: narrative describes the case of 2 adolescent patients who were incidentally diagnosed with the syndrome in the city of Cali Colombia at the imbanaco Clinic. information was obtained from their respective clinical histories, and ultrasound images and the respective surgical techniques used for the management of the symptoms were also extracted. the informed consent of both patients was obtained. Results: The diagnosis is usually late or incidental because the clinical presentation only becomes evident once menarche occurs. As the clinical and structural presentation of the pathology comprises a wide spectrum, therefore the diagnosis today is made by means of 2D and 3D ultrasonography, diagnostic imaging such as computed axial tomography and magnetic resonance imaging. the management will depend on the structural anomaly and the intensity of the symptoms. Today surgical method are usually the treatment of choice. Conclusion: OHVIRA syndrome is a rare congenital anomaly that represents a diagnostic challenge because of the regular menstruation and nonspecific clinical presentation . Health care providers should be aware of the different extending symptoms in order to employ different diagnose images such as 2D and 3D ultrasonography operated by highly trained staff (sensibility >90 % y specificity 100% to facilitate early and accurate management and improve patients quality life.