We report a case of a newborn presenting with severe compression of the spinal cord due to a large, solitary mass extending from C4 to T2. Neurosurgical exploration revealed a large intradural, extramedullary cystic lesion, compressing the spinal cord. Slowly progressive respiratory failure due to severe myelopathy led to the death of the child 19 days postpartum. At autopsy, a well-differentiated enterogenous cyst was found, the cyst wall containing gastric and esophageal type mucosa, and a bona fide muscularis propria. The gastrointestinal tract was completely normal. The possible developmental history of intradural enterogenous cysts is discussed.