Ectopic thyroid gland (ETG) is a rare entity. It may be located anywhere from the base of the tongue to mediastinum, and seldom in other sites. Most of the patients usually present with midline swelling which can be easily confused with other midline pathology, thereby leading to mismanagement in the form of surgical excision. The diagnosis of this entity requires clinical examination and different imaging modalities for proper management. Radionuclide studies play an important role in the diagnosis and further management of patients with ETG. In the present review we shall discuss various issues about location, embryology, clinical presentation and management including treatment of ETG.