Dandy-Walker malformation, also known as Dandy-Walker syndrome, is a rare congenital neurological anomaly that affects cerebellar development, the region of the brain responsible for motor coordination and balance. This posterior fossa anomaly is characterized by agenesis or hypoplasia of the vermis and cystic enlargement of the fourth ventricle, causing upward displacement of the tentorium and torcula. Most patients have hydrocephalus at the time of diagnosis. Dandy-Walker malformation is the most common posterior fossa malformation and typically occurs sporadically. The syndrome can manifest with a wide spectrum of neurological and developmental symptoms, making timely recognition and management crucial for improving patient outcomes. Treatment is primarily directed toward managing the clinical manifestations and associated comorbidities. This activity reviews the clinical presentation of Dandy-Walker malformation and highlights the interprofessional team’s role in its management.
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