Thyroglossal duct cyst(TGDC) commonly present as midline neck masses with or without infection. The presentation of TGDC at tongue base is quite uncommon in terms of location as well as presenting symptoms such as vomiting after feeding, stridor, chronic cough, upper airway obstruction, voice change, and dysphagia. Differential diagnosis should include lingual thyroid, dermoid cyst, vallecular cyst and other various tumors. Preoperative evaluation should include computerized tomography and thyroid scintigraphy scan. Though various surgical approcaches can be considered for TGDC of tongue base, Sistrunk procedure remains the standard, In emergent situation, aspiration of cyst may be helpful. We present a man with midline intraoral cystic swelling stretching from the base of the tongue and vallecular space. The mass was surgically removed by Sistrunk procedure and proven to be a TGDC.