Dandy-Walker syndrome(DWS) is a rare congenital disorder of the central nervous system,usually manifested by hydrocephalus. The typical anatomical features of DWS are hypoplasia of the cerebellar vermis, anteroposterior enlargement of the posterior fossa upward displacement of the sinus ring and transverse sinus, and cystic dilation of the fourth ventricle. Clinical symptoms can be manifested as a series of brain-related symptoms such as epilepsy, gait instability, speech dysfunction, mental symptoms and eye symptoms, and the clinical manifestations can exist alone or in combination. Current treatments include posterior fossa cyst resection, ventriculoperitoneal shunt, cyst peritoneal shunt, third ventriculotomy with or without choroid plexus burn under ventriculoscopy. The best treatment for DWS has been controversial for a long time, and there is a lack of comparison of large sample data, so a large number of randomized controlled trial are needed, This article aims to summarize the symptoms and treatments of DWS, and to provide reference for diagnosis and treatment options.