OBJECTIVE AND IMPORTANCENeurenteric cysts are very rare (particularly in the adult age group) congenital intraspinal cysts of endodermal origin. The patient described was a 48-year-old man who was diagnosed as having an isolated thoracic neurenteric cyst at the T5–T6 level, without a mediastinal enterogenous cyst. Radiological studies demonstrated multiple anterior vertebral column abnormalities.CLINICAL PRESENTATIONThe patient presented with a long history of mild midback pain. During the 2 years preceding admission, his midback pain worsened and he experienced right intercostal pain in the midthoracic area. Several months before admission, he noticed right lower extremity weakness, which led him to undergo neurological evaluation and radiological studies.INTERVENTIONThoracic spine x-rays revealed marked thoracic scoliosis and multiple vertebral abnormalities. Magnetic resonance imaging and myelography revealed an intradural extramedullary cystic mass at the T5–T6 level, severely compressing the spinal cord from the ventral side. Thoracic laminectomy was performed and the cystic lesion was completely removed, with disconnection of the ventral tract.CONCLUSIONThis is an unusual presentation, at this age, of a congenital intraspinal cyst in the thoracic area. Such cysts must investigated for early diagnosis and treatment. A neurenteric cyst should be suspected particularly if the cyst is associated with anterior vertebral column abnormalities.