Congenital syngnathia is a rare anomaly characterised by the presence of soft tissue (synechia) or bony adhesions (synostosis) between the maxilla and mandible. A wide spectrum and range of severity have been documented in case reports. Primarily it manifests in the inability to open the mouth; impacting on mandibular growth, nutrition, speech and management of the airway. The bony adhesions can be classified as partial or complete, as well as syndromic and non-syndromic. The aetiology of congenital syngnathia remains unknown. We describe a growth restricted, premature infant diagnosed postnatal with multiple congenital anomalies including syngnathia, craniosynostosis, ventriculomegaly, microcephaly, bilateral cataracts, facial dysmorphism, small kidneys, hypoplastic prepuce of the genitalia and bilateral syndactyly of toes 2, 3 and 4. The rare finding of syngnathia with the associated findings in a premature male infant weighing 1065 g at 31 weeks of corrected gestational age is the first to be reported.