Background Congenital anomalies of the urogenital system frequently coexist with renal abnormalities due to their shared origin from the intermediate mesoderm. Skeletal anomalies may also be associated, reflecting overlapping embryological development of mesodermal derivatives. Such multisystem involvement can pose a diagnostic challenge, particularly when identified incidentally. Case Presentation We report a 15-year-old female who presented with abdominal swelling and pain, later diagnosed as an umbilical hernia. During radiological evaluation, incidental findings of right renal agenesis, scoliosis, and a bicornuate uterus were identified. The patient underwent successful surgical management of the umbilical hernia. This case highlights the importance of recognizing coexisting anomalies and adopting an integrated approach in evaluation and management. Conclusion This case highlights the need for a systematic, bidirectional evaluation when a congenital anomaly is identified, particularly involving the renal, genital, and skeletal systems. A syndromic perspective is essential to avoid missed diagnoses, facilitate appropriate follow-up, and anticipate long-term implications in such patients.