The authors present a very rare case of ankyloglossia superior syndrome: first described in 1911 but with only 14 case reports in English literature it was of sufficient interest to discuss the aetiology, classification and management. As the palatal shelves fuse and elevate away from the tongue between 7th and 10th weeks gestation, synechiae may form between the tongue and palate. This abnormality has been described with lower limb abnormalities and may be part of a more general spectrum of abnormalities collectively termed oromandibular hypogenesis syndrome. Other craniofacial defects and gastrointestinal atresias are reported in association but the limb abnormalities appear the most common associated problem. The subject of the case report was successfully treated with a simple cold steel division of the synechiae, and normal feeding was established quickly. The authors manage to raise awareness of this rare condition and point towards multidisciplinary care for associated abnormalities but describe a simple procedure for the otolaryngologist to help establish normal tongue function.