Purpose <p>The aim of this study was to summarize and analyze the incidence, underlying causes and related risk factors of misdiagnosis in patients with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome.</p> Methods <p>This is a single center, retrospective study conducted in a tertiary hospital, enrolling patients diagnosed with OHVIRA syndrome in our center between January 2000 and December 2023, with intact charts retrieved. We collected information related to misdiagnosis. The main outcome was the incidence of misdiagnosis in OHVIRA patients. Misdiagnosis related information, including the primary diagnosis and following management were displayed. We also analyzed the potential risk factors of misdiagnosis in OHVIRA patients by logistic regression.</p> Results <p>Among 164 cases enrolled, 27 (16.5%) patients had experienced misdiagnosis. The most frequent primary misdiagnoses were pelvic abscess (8/27, 29.6%) and ovarian cyst (7/27, 25.9%). Complicating with pelvic endometriosis was the potential risk factor of misdiagnosis (OR 3.267, 95% CI 1.010–10.660). Presenting with irregular bleeding (OR 0.193, 95%CI 0.047–0.800) and performing pelvic MRI prior to diagnosis (OR 0.157, 95% CI 0.050–0.486) were potential protective factors for misdiagnosis in patients with OHVIRA syndrome.</p> Conclusion <p>Misdiagnosis is not uncommon in patients with OHVIRA syndrome. Alerting to related symptoms and optimizing various imaging modalities can effectively reduce the risk of misdiagnosis.</p>