The lingual cyst lined by respiratory epithelium is a rare pathology. It probably appears from the default of undifferentiated cells of the foregut during embryonic growth. This pathology is seen more often in males and children; however, only 5 patients younger than 4 years old have been reported. The pathophysiology and the management of this cyst were described in a 4-year-old girl. She presented with a soft mass on the dorsum of the tongue covered by normal mucosa, which existed since her birth, causing difficulty in eating, breathing, and talking. The magnetic resonance described a hyperintense image with an anteroposterior diameter of 27 mm, craniocaudal of 19 mm, and transversal of 26 mm in the midline groove of the tongue; the scintigraphy showed normality. The enucleation of the lesion was performed, eradicating the capsule of the cyst and obtaining a complete cleavage. The histopathologic examination defined a cyst lined predominantly by respiratory epithelium. Unlike in other cases, in this case their cystic lining and capsular constituents were contemplated considering the current histological recommendations. It is relevant to differentiate this pathology from other cysts with similar histological findings.