Notocord represents a primitive vertebral column which engenders development of prospective vertebrae and inevitably configures nucleus pulposus of the intervertebral disc. Ectopic remnants of notochord tissue were observed within the posterior clivus by Hubert von Luschka in 1856. “Ecchordosis physaliphora” as a terminology for denominating benign neoplasm arising from remnants of the notochord was introduced by Rippert in 1894. Ectopic notochordal rests are generally discerned at the clivus, base of skull or anterior pons. Ecchordosis physaliphora is a congenital, benign hamartoma-like neoplasm emerging as a derivative of remnants of notochord. As a congenital, benign hamartoma engendered from remnants of notochord, ecchordosis physaliphora commonly incriminates the retroclival prepontine region. Intradural chordoma may be misinterpreted and contemplated as an enlarged ecchordosis physaliphora. Nevertheless currently, chordoma, notochordal remnant lesions and ecchordosis physaliphora are considered to be distinct entities with a common genesis.