Monarch Disease Ontology term MONDO_0008740 (agnathia-otocephaly complex) can be described as follows. Agnathia-holoprosencephaly-situs inversus syndrome is an extremely rare and fatal association syndrome, characterized by absence of the mandible, cerebral malformations with facial anomalies related to a defect in cleavage in the embryonic brain (e.g. synophthalmia, malformed and low-set ears fused in midline (otocephaly), agenesis of the olfactory bulbs, microstomia, hypoglossia/aglossia) and situs inversus partialis or totalis. Also known as: agnathia-holoprosencephaly-situs inversus syndrome, dysgnathia complex agnathia-holoprosencephaly, holoprosencephaly-agnathia, otocephaly.