Purpose of Review <p>Discuss common and uncommon features of obstructed hemivagina with ipsilateral renal anomaly (OHVIRA), including diagnostic strategies, management, referral considerations, and the impact of OHVIRA on reproductive health throughout the patient’s lifespan. Coding and billing resources are also included to aid in accurately capturing the complexity of Müllerian anomalies.</p> Recent Findings <p>Two-thirds of patients with OHVIRA have endometriosis on laparoscopy. Patients with OHVIRA experience normal fertility rates comparable to the general population, but may be at increased risk for certain pregnancy complications such as malpresentation and preterm delivery. Recent reports indicate there may be risk for rare forms of cervical cancer on the previously obstructed side. Strategies for recognition and management may differ in high resource versus low resource settings.</p> Summary <p>OHVIRA is a complex Müllerian anomaly, and diagnosis is often delayed. High resolution MRI is preferred for most accurate pre-surgical diagnosis, however this imaging modality may not be available in low resource settings. Development of accessible advanced imaging tools may provide improved diagnostic capabilities in limited resource settings. Medical management is appropriate for many patients until a comprehensive plan including post-operative considerations is finalized. Surgical correction should be performed by a specialist or subspecialist with appropriate training. Long term reproductive health implications to consider include increased risk of endometriosis, certain pregnancy complications, and possible rare gynecologic cancer risks.</p>