Abstract
The Dandy-Walker syndrome (DWS) encompasses a group of anatomical midline cerebellar disorders with potential shared embryological origins, including the classic Dandy-Walker malformation, Blake's pouch cyst, and mega cisterna magna. Genetic factors, chromosomal abnormalities, and environmental influences contribute to its etiology. DWS, occurring in 1 in 25,000 to 35,000 live births, often presents with hydrocephalus and other central nervous system anomalies. Clinical manifestations vary, with symptoms appearing from neonatal to adult stages. Diagnosis is performed through neuroimaging, evaluating the posterior fossa and associated anomalies. Management involves treating hydrocephalus, addressing associated anomalies, and providing neurological follow-up with a multidisciplinary team. Prognosis hinges on associated malformations and their severity, impacting long-term outcomes. An unsystematic updated review on the embryology, pathophysiology, diagnostic approach, and therapeutic management of DWS is presented.