Congenital hernias in the lumbar region are very uncommon. Approximately 10 per cent of all lumbar hernias are congenital and the vast majority are unilateral. We report our experience with seven congenital lumbar hernias in six infants treated during a five year period. All patients presented in the first year of life with abnormal protrusions in the lumbar region. In addition to the lumbar hernia, major associated malformations, including caudal regression anomalies, diaphragmatic hernia, ureteropelvic junction obstruction, cloacal exstrophy and lipomeningocele, were observed in 66 per cent of the infants and were consistent with lumbocostovertebral syndrome. In five patients, unilateral defects were repaired primarily. One patient with bilateral lumbar hernia underwent staged repair. The larger right-sided defect was repaired using a polytetrafluoroethylene prosthesis. At a second operation two months later, the small left-sided hernia was closed primarily. All patients have done well without recurrence with a follow-up period ranging from four to 48 months. Early repair of congenital lumbar hernias in infants after correction of other life-threatening conditions is advocated. Unlike the acquired variety, congenital lumbar hernia may include a more extensive deficiency of the entire lateral abdominal wall extending to the rectus sheath and inguinal ligament and satisfactory closure of the defect without prosthetic material may be difficult or impossible.