The human tail has been intermittently described in the literature since the early 1900s. These have typically been isolated cases presented primarily with intrigue and medical curiosity. Presented here is a series of 6 neuroectodermal appendages with a proposal for their etiological development. The material presented will support a theory of the superficial extension of a dermal sinus tract in the formation of neuroectodermal appendages. These are characterized by: a posterior localization in or near the midline, a tubular or ‘tail-like’ appearance, extension of the appendage into the spinal canal with attachment to neural elements, variable vertebral defects and occasionally an associated appendage which may appear as either a separate entity (probably due to breakage during development) or in connection with the posterior appendage. The appropriate evaluation and treatment of this entity will also be discussed.