We report the first clinical case of a term infant with findings suggestive of lumbocostovertebral syndrome and VACTERL association. Renal agenesis was suspected by prenatal ultrasound, and at birth clinical findings included a large abdominal wall hernia, imperforate anus, vestibular fistula, absent right rib, vertebral anomalies, tethered cord, syringomyelia, and two small sacral dimples.