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. 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. We report a clinical case of a Dandy-Walker congenital malformation in a young patient in the only neurology department in Bangui, Central African Republic. Through this observation, and in a challenging medical practice context, we draw the attention of healthcare providers to the importance of medical imaging (cerebral computed tomography) in the face of neurological deficits, signs of intracranial hypertension, and/or hydrocephalus in young patients and children.
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