Orofaciodigital syndrome is a rare congenital malformation characterised by anomalies such as microretrognathia, cleft/lobulated tongue, cleft palate, lingual hamartoma and postaxial polydactyly of the hands. OFD14, a subtype of orofaciodigital syndrome, also presents with severe intellectual disability and microcephaly as well as vermian hypoplasia with the molar tooth sign on brain imaging studies.