The most common craniofacial disorders in pediatric patients are congenital disorders, and there are many ways to classify them, among which the DeMyer classification is the most widely used. DeMyer is a classification of pediatric craniosynostosis into three major categories, namely, diseases of cellular origin, diseases of tissue origin, and diseases of organ origin. In addition, there are some diseases whose etiology is not yet clear, making it difficult to make a classification. DeMyer’s classification, which is widely accepted by scholars in the field, specifically classifies pediatric congenital disorders into 8 major categories: 1. Congenital intracranial tumors. Here, congenital tumors are a type of intracranial tumors that are present at birth. They are usually present before birth, but the age of onset can vary. The most common clinical congenital intracranial tumor is craniopharyngioma, followed by epidermoid cyst, dermatofibrosarcoma and teratoma. 2. Neurocutaneous syndrome. It refers to lesions mainly involving the nervous system and skin organs. Under normal circumstances, the neural tube matures during early embryonic development, then evolves into the brainstem and brain, and the ectoderm evolves into skin tissue. If the ectoderm develops abnormally during this process due to genetic or other factors, it can cause neurocutaneous syndromes that involve both neural and skin tissues. The common ones are neurofibromatosis, tuberous sclerosis and cerebral facial angiomatosis, all of which are autosomal dominant disorders. 3. Congenital cerebrovascular disease. Congenital cerebral aneurysm can be caused by dysplasia of the middle and outer layers of the cerebral arterial blood vessel wall, which is the most common one. In addition, there are intracranial vascular malformations, smog, congenital internal carotid artery deficiency and congenital internal carotid artery bending and kinking syndrome. 4.Disorders of neural tube closure. The congenital craniosynostosis caused by neural tube closure disorder is the most common type of congenital malformation of the cranial brain. In the 3rd-20th weeks of gestation, the fetal neural tube development defect or atresia leads to such malformations and has family heritability. These include craniosynostosis, meningeal bulge, anencephaly, empty cranial cavity, hypoplasia or absence of corpus callosum, lipoma or teratoma of the corpus callosum, and Chiari malformation and Dandy-Walker syndrome. 5. Diverticulogenic disorders. The fetal primitive forebrain does not divide normally to form telencephalon, mesencephalon and ventricular system in the 4th-8th week, it will cause a series of complex cranial malformations such as anencephaly, forebrain anencephaly, etc. 6.Neuronal proliferation disorder. Too much or too little neuronal proliferation causes macrocephaly and microcephaly. 7.Neuronal displacement disorder. Neuronal translocation disorder can occur in the 12th-24th weeks of gestation, resulting in abnormal position of brain tissue, which can lead to anencephaly and foramen ovale malformation. If neuroblastoma cells do not move to the cortical surface in time, gray matter heterotopia can occur. 8. Destructive lesions. Various destructive factors during pregnancy and delivery can lead to fetal brain damage. Such as infection, poisoning and hypoxia can cause hydrocephalic anencephaly or cerebral penetration malformation. By interpreting the DeMyer classification, which is currently widely used, we have a more comprehensive understanding of pediatric congenital craniosynostosis and provide scientific theoretical support for the diagnosis and treatment of these diseases.