Congenital lumbar hernia is a rare disease affecting mainly infants. Its association with lumbo-costo-vertebral syndrome (LCVS) is hardly seen in the literature. We present a case of LCVS in a 1-month-old female infant presenting with a soft bulge in left lumbar region. Abdominal X-ray revealed absence of multiple ribs with a mild lumbar scoliosis and defective ninth vertebra. Ultrasonography showed absence lateral abdominal wall muscles in left lumbar region and 2.5 cm diameter lumbar hernia. Primary repair of the adnominal wall defect was performed without meshplasty and no recurrence was reported. We hope to enhance the literature of this rare disease with our case.
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