This review encompasses seven patients with clinically important cystic lesions of the gastrointestinal (GI) tract, exhibiting a wide range of vertebral anomalies and connections to the neural canal. Three patients had mediastinal masses connected to lower cervical and upper thoracic anomalous vertebrae with intraspinal extensions. In addition, one of these patients had a separate, juxtapancreatic intestinal duplication cyst. One infant with colonic duplication had a lumbar vertebral anomaly and an epithelial-lined tract between the two. Another patient had a presacral cystic mass which was the site of recurrent infections and meningitis until a connection with the rectum was divided. A newborn baby had a completely split notochord syndrome with a large dorsal enteric fistula. Finally, one patient had a dorsal enteric cyst with a direct intraspinal connection. Four of the seven patients had associated significant congenital anomalies, two of whom died early in the neonatal period. The rest of the patients did well. This broad range of enteric lesions with associated vertebral and intraspinal abnormalities suggests that the clinical spectrum of neurenteric cystic lesions is much wider than is generally appreciated.