An intraspinal cervicodorsal endodermal epithelial cyst occurred in a 20-year-old man who experienced sudden paraparesis at the age of 6 years and subsequently developed progressive sensory disturbances. The patient underwent repeated surgical explorations indicating that the lesion may be difficult to identify. The cyst wall consisted of the outer smooth muscle and connective tissue and the inner epithelium that produced mucin and mucopolysaccharide. The intimate association between the epithelium and smooth muscle makes the cyst wall strikingly like the wall of the digestive tract or the respiratory passage and supports the endodermal origin of the cyst which must be distinguished from the neuroepithelial cyst. The pathogenesis of the endodermal epithelial cyst is suggested to be related to congenital maldevelopment of the notochord and the primitive gut.