Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital anomaly that affects females. Although it is possible to diagnose in early life, most cases are usually diagnosed in adolescence or adulthood. This delay in diagnosis can cause a variety of complications, some associated with infertility or renal failure. Here, we report the case of a female child with unilateral renal agenesis identified on the prenatal scans. Prompt postnatal imaging made it possible to diagnose the concomitant gynecological malformation during the first year of life. This report highlights the importance of an early diagnosis in such cases, which could be done through routine screening for gynecological anomalies in patients with prenatal diagnosis of renal malformations.