Gastrointestinal cyst found within the oral cavity has been reported as a rare occurrence with less than 40 cases. The tongue is the preferred site with almost 60% of cysts occurring within oral cavity. The cyst usually shows an asymptomatic swelling in the floor of the mouth, about 30% of affected individuals have symptoms related to difficulties with feeding, swallowing and respiration. The histopathologic features of the cyst vary with respect to the type of epithelium lining; gastric, squamous, intestinal and respiratory epithelium. The pathogenesis is not known, but it may be related to entrapment of undifferentiated endoderm within oral cavity during early fetal development. In this article, we report a case of a gastrointestinal cyst of the tongue in a 16-month-old girl.