Enterogenous cyst is a rare cause of spinal cord compression. It is resulted from dysembriogenetic error developed at the third week of the embryo life. A case of 17-year-old male with an intradural extramedullary enterogenous cyst of the cervical cord is presented. In appeared as a mass of low-intensity signal in comparison with the spinal cord on magnetic resonance imaging. Histologically, it was confirmed by epithelium which was composed of single pseudostratified mucus-secreting cuboidal or columnar cell with hyalinized fibrous wall. Total removal was possible without complications. The clinical and radiological findings and it's morphogenesis are discussed with a review of the literature.