Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex of structural abnormalities of the female urogenital tract. A 17-year-old girl with uterine didelphys associated with OHVIRA syndrome presented with progressive development of cyclic lower abdominal discomfort and a large abdominopelvic mass. We describe the findings from ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), the first case report of this syndrome to examine all three different imaging modalities in a single patient. We also review the literature on OHVIRA syndrome and discuss important considerations relevant to radiologists and other clinicians.