Split notochord syndrome is a rare neural tube malformation involving the brain, spinal cord, and vertebral column. In nearly half of the cases, the malformation also involves the gastrointestinal tract in the form of a dorsal neurenteric fistula and imperforate anus. In the literature, less than 50 cases have been reported. To the best of my knowledge, this is the first reported case associated with a left congenital diaphragmatic hernia.