Obstructed hemivagina with an ipsilateral renal anomaly (OHVIRA) syndrome is a congenital malformation that presents as a uterine didelphys with an obstructed hemivagina and an associated ipsilateral renal aberration. The clinical symptoms usually manifest after menarche. Unlike the typical presentation in adolescence, this case report features a neonatal presentation of OHVIRA syndrome with an unusual renal association. A female twin delivered at 35 weeks of gestation was transferred to our institution after birth from an outside hospital due to respiratory distress and for evaluation of the left multicystic dysplastic kidney identified on prenatal ultrasound. Physical examination and lab results, including a complete blood count, and a basic metabolic panel, including blood urea and serum creatinine, were within the normal range for age. Abdominal and pelvic ultrasound showed multicystic dysplastic left pelvic kidney, congenital hepatic cyst measuring 6 mm, uterine didelphys with duplication of the vaginal canal, and obstructed left hemivagina corresponding to the OHVIRA syndrome. Further testing revealed a normal chromosomal microarray, small patent foramen ovale on the echocardiogram, no vertebral or rib anomalies on the spinal x-ray, normal hearing test, and mild optic cupping on the ophthalmological evaluation. The pediatric surgeon and urologist recommended an outpatient follow-up and elective surgery in the future. This is a unique case presenting in the neonatal period with an unusual association. Timely intervention can help prevent obstetric complications.