VACTERL is an acronym for Vertebral anomalies, Anal atresia, Congenital cardiac disease, Tracheo-esophageal fistula, Renal anomalies, and Limb defects. VACTERL associated with hydrocephalus has rarely been reported and is thought to be an autosomal recessive anomaly. The condition is described as a uniformly lethal or developmentally devastating disorder distinct from the VATER association.