Background. Neuroglial choristomas are rare entities that are composed of differentiated neuroectodermal cells presenting where they do not belong.

Case. Here in this paper, we represent a two-year old patient with a very rare presentation of neuroglial choristoma which manifested itself within a thyroglossal duct cyst. In this paper we will also discuss pathogenesis, clinical manifestation, differential diagnosis and management of the neuroglial choristomas.

Conclusion. In conclusion we believe this unique case may aid in understanding the pathophysiology, differential diagnosis, and management of this rare congenital anomaly.

Keywords: choristoma, neuroglial, sistrunk, thyroglossal duct