Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital abnormality of the female urogenital tract. We reported OHVIRA syndrome in a 15-year-old girl, who was referred to King Faisal Specialist Hospital and Research Centre, due to hematocolpos and an imperforate hymen. The patient was diagnosed as having an absent right kidney since childhood. She presented with progressive development of lower abdominal discomfort and an abdominopelvic mass. She had no history of urinary complaints. Here, we describe our findings from the abdominal ultrasound and magnetic resonance imaging.