Children with omphalocele, exstrophy, imperforate anus, and spinal defects complex present with the most severe form of birth defects in the exstrophy-epispadias spectrum. Prenatal diagnosis is difficult, but improved survival over the past several decades makes understanding the potential anatomic manifestations imperative for expeditious and appropriate surgical care. The upper urinary tract is often normal in children with omphalocele, exstrophy, imperforate anus, and spinal defects complex, but malposition of one of the kidneys has previously been reported. We present the first case of bilateral kidney herniation into the omphalocele sac.