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Background: Dandy-Walker Syndrome (DSW) is a complex malformation that involves the posterior fossa and the cerebellum, being considered a rare condition with an estimated incidence of 1 in 30,000 births. Hydrocephalus is its most common finding, identified in approximately 80% of cases, and SDW is present in between 4% and 12% of cases of hydrocephalus in infants. The "classical triad" of SDW is defined by complete or partial agenesis of the vermis, enlargement of the posterior fossa with upward displacement of the tentorium, transverse and torcular sinuses, and cystic dilatation of the fourth ventricle. Objective: To report a case of SDW attended in our Service. Method: This is a single case report treated at the Gynecology and Obstetrics Service of the São Francisco University Hospital in Providência de Deus - HUSF, located in the city of Bragança Paulista, SP, Brazil. The patient authorized the use of information about the case by signing an informed consent form. Conclusion: This report demonstrated the importance of imaging techniques, such as magnetic resonance imaging, ultrasound, for the identification, even during the prenatal period, of fetuses with the syndrome, allowing the medical team to design the treatment suitable for the future newborn. The findings provided by the imaging analyzes allowed us to investigate in great detail all the changes indicative of SDW after the 18th week of gestation, as well as after delivery. [ABSTRACT FROM AUTHOR]

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