Congenital neural tube anomaly is a defect in the formation of the neuroembryo and is the most common birth defect with an incidence of 0.6 to 3.7 per 1000 live births. The pathogenesis includes both genetic and environmental factors. Possible environmental factors include geographic location, season of conception, socioeconomic level, maternal age, zinc and folic acid deficiency, maternal alcohol abuse, and maternal use of antiepileptic drugs. And genetics determines the susceptibility to environmental factors. They are classified as open and closed according to neural tissue exposure or not. Open neural tube malformation: neural tissue is exposed due to defective formation of primary neuroectoderm with cerebrospinal fluid leakage, e.g., with hydrocephalus, Chiari II malformation. Closed neural tube malformation: Due to defective secondary neurogerm formation, neural tissue is not exposed and the defective area is covered with dysplastic skin tissue, including: anencephaly, cerebrospinal bulge (meningeal bulge or spinal cord bulge), craniospinal bifida, congenital dermal sinus, open spina bifida, spinal cord spinal herniation, meningeal bulge, spina bifida, spinal cord longitudinal bifida, caudal hypoplasia, etc. Open neural tube defects are visible at birth and the vast majority can be detected during gestation. Neural tube defects can cause progressive neurodegeneration, with symptoms appearing shortly after birth or later in life, due to concomitant hydrocephalus, Chiari II malformation, cystic masses of the spinal cord tethering, or fibrous bands compressing the neural tissue, which can be complicated by meningitis. Closed neural tube malformations have different presentations and can also have no skin markings, making them unrecognizable for many years. The most common manifestations are distinct abnormalities along the spine, such as fluid-filled cystic masses, hypopigmented, or hyperpigmented areas, dermal hypoplasia, congenital dermal sinuses, small hairy patches, skin appendages, and asymmetric gluteal clefts. Closed neural tube defects. This is followed by asymmetry of the lower extremities or feet, with one limb being thinner, smaller feet, high arched feet or claw toes. Other children present with progressive spinal deformities such as scoliosis. Some children present with progressive neurological deficits, including weakness of one distal lower extremity, sensory deficits, and bladder and rectal dysfunction, which are associated with spinal cord tethering. MRI is preferred for suspected neural tube deformities and can detect spinal or intracranial malformations. CT scans can show bony defects and anatomy and facilitate the detection of hydrocephalus or other intracranial malformations. Clinical manifestations Lumbosacral skin changes: bulging or depressed lumbosacral skin, possibly with secretions or infection; hypertrichosis; occult spina bifida, dermatomal sinus, spondylolisthesis, subcutaneous lipoma, etc. Manifestation of abnormal walking, weakness of lower limbs, ankle deformation (clubfoot). Manifestation of abnormal sensation and pain in the lower extremities, perineum and low back. Open neural tube deformity surgical treatment includes: early closure of the defect; ventriculoperitoneal shunt with closure of the defect in children with hydrocephalus; suboccipital posterior cranial fossa decompression in symptomatic Chiari deformity; early tethering release in children with spinal cord tethering.