Glial heterotopia of oropharynx is a congenital anomaly, whereby ectopic mature glial tissue is found around oropharynx isolated from the brain and spinal cord. Herein we report a rare presentation of a mass at the base of tongue in a neonate. In addition, to underscore the rarity of oropharygeal glial heterotopia, we discuss the dilemma in approaching its diagnosis and management in a neonate.