Dorsal enteric cysts have been recognized in association with a wide variety of developmental anomalies of the back, the spine, the central nervous system, the mediastinum and the gut. These lesions can be lethal and they caused the deaths of three of the eight patients in this study: two from meningitis and one from erosion into the aorta. Two of these deaths might have been prevented if they had been fully investigated earlier and the life‐threatening components of these complex lesions removed. In the most recent case, however, the lesion was detected antenatally, expediting postnatal investigation and surgery. Three of the five survivors have neurological sequelae attributable to their intraspinal patholog