Split notochord syndrome presenting with meningomyelocoele and dorsal enteric fistula

Split notochord syndrome is a rare congenital anomaly. We present the history of a 16-day-old male infant who presented to us with a lumbosacral meningomyelocoele and a dorsal enteric fistula along with the cleft of lower vertebrae. Review of the literature, has revealed eight such cases with very few survivals. We successfully treated the patient.