Congenital crooked mouth crying syndrome is a special face with congenital anomalies, mostly with eye and ear anomalies and congenital heart disease, such as ductus arteriosus, ventricular septal defect, atrial septal defect, etc., with congenital heart disease is more common. Therefore, if you find this face clinically, you should pay attention to whether there is “congenital heart disease”. The diagnosis can be made by facial electromyography to exclude facial palsy, because the crooked mouth is not caused by facial palsy, but by facial muscle dysplasia. The child’s facial expression has a special phenomenon: the lips are symmetrical to the left and right when he or she is smiling, but the corner of the mouth is pulled down when he or she cries, resulting in a crooked mouth and crying face. The cause is not due to birth injury or fetal malposition, and there is no facial nerve palsy on electromyography, but due to hypoplasia of one side of the orofacial descending muscle, which cannot pull down when crying, while the healthy side of the orofacial descending muscle still pulls down, resulting in an asymmetrical crying mouth. Clinical manifestations In the 1990s, it was discovered that most patients with congenital crooked mouth crying syndrome had a microdeletion of chromosome 22q11 (22q11.2), a chromosomal alteration associated with genetics, genetic mutations, intrauterine fetal infections, maternal diseases and medications. Some of the affected children have mothers with chronic alcoholism and diabetes mellitus. Due to this chromosomal abnormality, the 1st, 2nd, 3rd and 4th pairs of pharyngeal cervical neural crest cells migrate and distribute abnormally during fetal development, and the migration, fixation and distribution of nerves is a process involving multiple genes, so clinically there are complex and diverse malformations of the orofacial descending muscle, mouth, palate, ear, heart, thyroid and other multi-organ systems. Cardiovascular malformations are particularly common. Cardiovascular malformations may include tetralogy of Fallot, patent ductus arteriosus, tricuspid atresia, ventricular septal defect, etc. Because congenital crooked cry is closely associated with congenital malformations, it has received increasing attention in recent years. The incidence of chromosome 22q11 microdeletion is high, about 1 in 4000 live births, and its clinical manifestations are diverse, either with psychomotor disorders, developmental delays, or seizures, some of which can be concluded only through long follow-up. The congenital crooked mouth crying syndrome is not usually taken seriously by parents because it only changes when crying, and older children are more secretive because they cry less, so it is mostly detected in the treatment of other diseases, and there are limitations to the understanding of this phenomenon. Pediatricians should pay attention to the facial expressions of children in clinical work, and pay attention to the congenital crooked mouth crying sign to avoid missing the diagnosis, so that congenital anomalies can be detected in time and appropriate medical intervention can be given.