IntroductionThe most mobile organ of the body, the tongue is associated with various congenital anomalies; most of which are in association with many other systemic abnormalities. Rarely do they occur in isolation. Isolated aglossia, that we presented, is one of the more rare presentations.Case presentationOur patient is a 6-year-old male child of Asiatic origin from Kashmir (India), who was physically well built and mentally sound and presented with history of impaired speech. The patient had normal velopharangeal competence but absence of tongue which was replaced by a small mucus membrane projection near the floor of oropharangeal isthmus. The patient had no difficulty in feeding or taste sensation but he was unable to pronounce lingual consonants.ConclusionIsolated aglossia is very rare condition explained on the basis of growth failure of lateral lingual swellings and tubercular impar. Such patients do not usually need reconstruction of tongue; as feeding, swallowing and taste sensations are usually intact and speech cannot be improved by reconstruction. However, malocclusion of teeth needs to be taken care of.