The oromandibular–limb hypogenesis syndrome is a rare complex of jaw and limb defects with unclear aetiology. Multiple and variable deformities of the mandible, maxilla and tongue may occur in combination with a variety of limb defects. The wide range and combination of anomalies make classification difficult. A patient with all of the features of oromandibular–limb hypogenesis syndrome (except cranial nerve palsy) is presented. Despite severe micrognathia, synostosis of the jaws and soft-tissue synechiae between the hypoplastic tongue and the cleft palate, this patient had no respiratory difficulty and thrived with nasogastric feeding. The surgical management is discussed with emphasis given to the difficult airway and restoration of normal function of the tongue and jaws.