Neuroglial heterotopia (NGH), is a displaced mass of mature central neuroepithelial tissue unconnected to the brain proper. NGH presents mostly with respiratory distress, neck mass and feeding difficulties at early age.In our case, the nasopharyngeal NGH, a nine years old female child presented with otalgia and reduced hearing of left ear. Otological examination showed secretory otitis media. On nasal endoscopy solitary, smooth, mucosa covered mass on the eustachian tube opening of left side blocking eustachian tube orifice. Imaging showed a well-defined, non-enhancing, isodense soft tissue lesion arising from left lateral wall of nasopharynx with complete opacification of middle ear and mastoid. Patient underwent left myringotomy and grommet insertion. Nasopharyngeal mass removed trans-nasally under endoscopic guidance. Histopathological examination of the mass showed features of Neuroglial heterotopia. Patient improved symptomatically.Possibility of NGH should be considered while evaluating children with congenital nasopharyngeal mass. Thorough clinical evaluation, radiological imaging to rule out intracranial connection and complete excision are necessary for successful management of such cases.