Background: Congenital lumbar hernia, a rare type of hernia, can present in association with lumbocostovertebral syndrome, involving multiple congenital anomalies such as rib anomalies, scoliosis, and hemivertebrae. These hernias, which protrude through either the superior or inferior lumbar triangles, can pose significant diagnostic and therapeutic challenges. Case Report: A one and a half-month-old baby boy presented with a progressively enlarging mass in the right flank region since birth, becoming more pronounced during crying. Surgical repair involved reducing the hernial sac contents, defining the defect edges, and reinforcing the area with a 5×4 cm polypropylene mesh. The post-operative period was uneventful, and the patient was discharged on post-operative day 4. Conclusion: Congenital lumbar hernia with lumbocostovertebral syndrome is a rare but critical condition in pediatric patients. Early diagnosis and prompt surgical intervention are imperative to prevent complications. Open prosthetic repair remains the preferred method, providing reliable and effective results. Awareness of associated congenital anomalies is crucial for comprehensive patient management.