Background: Lumbo-costo-vertebral syndrome is a set of rare abnormalities involving vertebral bodies, ribs, and abdominal wall.
Case: We present a case of Lumbo-costo-vertebral syndrome in a 6-month old female infant who had a progressive swelling over the right lumbar area since birth. Clinical examination revealed a reducible swelling on the right flank with positive cough impulse. Ultrasonography showed a defect containing bowel loops in the right lumbar region. Chest X-ray revealed scoliosis and hemivertebrae with absent lower ribs on the right side. Computer tomography scan showed hernia sac containing the bowel and the right lobe of the liver with cross fused kidney.
Conclusion: Lumbo-costo-vertebraly syndrome is a rare condtion which could be associated with different organ malformations. Simple closure or meshplasty could be done depending on the size of the defect.
Keywords: hemivertebrae; lumbar hernia; lumbo-costo-vertebral.