Based upon the observation of four cases of ectopic lingual thyroid the authors discuss the etiopathogenetic, clinical-diagnostic and therapeutic aspects of this pathology. The clinical characteristics of the mass, scintigraphy, echography and CT scan of the neck, the T3, T4, TSH doses in the blood and fine needle aspiration of the mass have made adequate appraisal of its possible nature and have led to the identification of the presence of thyroid tissue in other sites. In regard to therapy, the authors believe that surgery is compulsory for patients showing clinical signs of upper airway obstruction despite suppressive therapy or when the lesion shows signs of malignant degeneration. After having mentioned the more commonly employed surgical approaches, emphasis is placed on median pharyngotomy as it permits the best visualization of the region, a careful excision of the mass and a valid control over bleeding. In most cases a tracheotomy proves unnecessary. The post surgical hormonal situation suggests the opportunity of a possible substitutive therapy with synthetic hormones or thyroid derivatives.