The split spinal cord is a rare congenital malformation. We report the rare finding of a split cord malformation in a young girl. Further evaluation of this anomaly revealed a Type I split cord malformation (midline bony septation), with no other concomitant pathological entities. Various hypotheses have been made regarding the embryology of this unusual form of spinal dysraphism, and these are reviewed along with the common clinical manifestations of this intriguing pathological entity. © 2005 Wiley-Liss, Inc.