Congenital Lumbar hernia was previously known as Lumbocostovertebral syndrome. This syndrome consists of the presence of hemivertebrae, rib defects, abdominal wall anomalies and hypoplasia of the abdominal musculature. This case report describes a 40-hour-old infant with malrotation of left kidney and hydronephrosis in association with congenital lumbar hernia. Various congenital renal anomalies like renal pyelectasis, bilateral renal agenesis and pelviureteric junction obstruction, malascended kidneys, hypospadiasis have been reported in association with this disease. Malrotation has only been reported in adults so far. However ours is the first ever case in literature to report malrotation of kidney in association with this syndrome in neonatal age group. Treatment of this condition is done usually by mesh hernioplasty. Presence of this condition must prompt the surgeon to carry out all possible investigations to rule out various other congenital orthopedic, neurological, and urological anomalies.
Keywords: Lung hypoplasia; Malrotation; Renal anomaly; Scoliosis.