INTRODUCTION: OHVIRA-syndrome (obstructed hemivagina, ipsilateral renal agenesis/anomaly) is a rare Mullerian duct anomaly that can lead to complications in pubescent children. CASE REPORT: We report a case of a 13-year-old patient with acute right-sided lower quadrant abdominal pain who was referred for exclusion of appendicitis. As a result of the examination (transvaginal ultrasound scan and gynecological examination), a female genital tract anomaly was suspected in the form of obstructed hemivagina with hematocolpos and hematometra. The MRI scan showed hematocolpos and hematometra on the right side, uterus didelphys accompanied by right-sided renal agenesis, consistent with OHVIRA-syndrome. Excision of the vaginal septum was performed and the accumulated old menstrual blood, as represented by hematocolpos and hematometra, was evacuated. Postoperative recovery was uneventful. CONCLUSION: The early surgical management of this rare Mullerian duct anomaly is important in order to prevent longterm complications. This malformation should be considered in the differential diagnosis of acute lower abdominal pain in pubescent girls.